Exogenous material on, under, or embedded within the conjunctiva or sclera; commonly dirt, glass, metal, or cilia. Patients usually note foreign body sensation and redness; may have corneal staining, particularly linear vertical scratches due to blinking with a foreign body trapped on the upper tarsal surface. Good prognosis.

• Remove foreign body; evert lids to check tarsal conjunctiva.

• Topical broad-spectrum antibiotic (polymyxin B sulfate-trimethoprim [Polytrim] drops or bacitracin ointment qid).

Laceration

Partial-thickness or full-thickness cut in conjunctiva with or without partial-thickness cut in the sclera; very important to rule out open globe (see below); good prognosis.

• Seidel test for suspected open globe (see below).

• Topical broad-spectrum antibiotic (gatifloxacin [Zymar] or moxifloxacin [Vigamox] qid).

• Conjunctival and partial-thickness scleral lacerations rarely require surgical repair.

Open Globe

Full-thickness defect in eye wall (cornea or sclera), commonly from penetrating or blunt trauma; the latter usually causes rupture at the limbus, just posterior to the rectus muscle insertions, or at previous surgical incision sites; double penetrating injuries are called perforations; an open globe may also be due to corneal or scleral melting.

Associated signs include lid and orbital trauma, corneal abrasion or laceration, wound dehiscence, positive Seidel test (see Laceration section in Chapter 5), low intraocular pressure, flat or shallow anterior chamber, anterior chamber cells and flare, hyphema, peaked pupil, iris transillumination defect, sphincter tears, angle recession, iridodialysis, cyclodialysis, iridodonesis, phacodonesis, dislocated lens, cataract, vitreous and retinal hemorrhage, commotio retinae, retinal tear, retinal detachment, choroidal rupture, intraocular foreign body or gas bubbles, and extruded intraocular contents. Guarded prognosis.

Figure 4-1 (A) Conjunctival foreign body, demonstrating a fragment of corn husk embedded in the conjunctiva. (B) Conjunctival foreign body, demonstrating a grasshopper leg embedded in the conjunctiva. (C) Same patient as in (B), demonstrating multiple vertical corneal abrasions from a grasshopper leg in the superior tarsal conjunctiva. Such a pattern of linear abrasions suggests a foreign body under the upper eyelid, and therefore the examiner should always evert the eyelid to inspect for this.

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Figure 4-2 Conjunctival laceration with gaping edges.

Figure 4-3 Full-thickness corneoscleral limbal laceration with wound gape. Note discontinuity of slit-beam as it crosses the wound edge (arrowhead).

Figure 4-4 Penetrating injury with foreign body (nail) protruding from globe.

Figure 4-5 Open globe. There is a temporal full-thickness scleral laceration with uveal prolapse. Also note the extensive subconjunctival hemorrhage and upper and lower eyelid lacerations.

OPHTHALMIC EMERGENCY

• Admit for surgical exploration and repair; protect eye with metal eye shield; minimize ocular manipulations; examine globe only enough to verify the diagnosis of an open globe; remainder of examination and exploration should be performed in the operating room. Postoperatively, start antibiotics and steroids.

• Consider B-scan ultrasonography if unable to visualize the fundus.

• Consider orbital computed tomography (CT) scan or orbital radiographs to rule out intraocular foreign body; magnetic resonance imaging (MRI) is contraindicated if foreign body is metallic.

• Subconjunctival antibiotics and steroids:

Vancomycin (25 mg).

Ceftazidime (50–100 mg) or gentamicin (20 mg).

Dexamethasone (12–24 mg).

• Topical fortified antibiotics (alternate every 30 minutes):

Vancomycin (25–50 mg / mL q1h).

Ceftazidime (50 mg / mL q1h).

• Topical steroid (prednisolone acetate 1% q1–2 h initially) and cycloplegic (scopolamine 0.25% or atropine 1% tid).

• Systemic intravenous antibiotics for marked inflammation or severe cases:

Vancomycin (1 g IV q12h).

Ceftazidime (1 g IV q12h).

• Small corneal lacerations (< 2 mm) that are self-sealing or intermittently Seidel positive may be treated with a bandage contact lens, topical broad-spectrum antibiotic (gatifloxacin [Zymaxid] or moxifloxacin [Vigamox] q2h to q6h), cycloplegic (cyclopentolate 1% bid), and an aqueous suppressant (timolol maleate [Timoptic] 0.5% or brimonidine [Alphagan-P] 0.15% bid); observe daily for 5–7 days; consider suturing laceration if wound has not sealed after 1 week.

Subconjunctival Hemorrhage

Diffuse or focal area of blood under the conjunctiva. Appears bright red, otherwise asymptomatic. May be idiopathic, associated with trauma, sneezing, coughing, straining, emesis, aspirin or anticoagulant use, or hypertension, or due to an abnormal conjunctival vessel. Excellent prognosis.

• Reassurance if no other ocular findings.

• Consider blood pressure measurement if recurrent.

• Medical or hematology consultation for recurrent, idiopathic, subconjunctival hemorrhages, or other evidence of systemic bleeding (ecchymoses, epistaxis, gastrointestinal bleeding, hematuria, etc.).

Figure 4-6 Subconjunctival hemorrhage demonstrating bright red blood under the conjunctiva. As the hemorrhage resorbs, the edges may spread, become feathery, and turn yellowish (arrowhead).

Telangiectasia

Definition

Abnormal, dilated conjunctival capillary formation.

Symptoms

Asymptomatic red spot on eye; patient may have epistaxis and gastrointestinal bleeding depending on etiology.

Signs

Telangiectasia of conjunctival vessels, subconjunctival hemorrhage.

Figure 4-7 Conjunctival telangiectasia appearing as dot-like, corkscrew, irregular vessels near the limbus.

Differential Diagnosis

Idiopathic, Osler–Weber–Rendu syndrome, ataxia–telangiectasia, Fabry’s disease, Sturge–Weber syndrome.

Evaluation

• Complete ophthalmic history and eye exam with attention to conjunctiva, cornea, lens, and ophthalmoscopy.

• Consider CT scan for multisystem disorders.

• Medical consultation to rule out systemic disease.

Management

• No treatment recommended.

Prognosis

Usually benign; may bleed; depends on etiology.

Microaneurysm

Definition

Focal dilation of conjunctival vessel.

Symptoms

Asymptomatic; may notice red spot on eye.

Signs

Microaneurysm; may have associated retinal findings.

Differential Diagnosis

Diabetes mellitus, hypertension, sickle cell anemia (Paton’s sign), arteriosclerosis, carotid occlusion, fucosidosis, polycythemia vera.

Evaluation

• Complete ophthalmic history and eye exam with attention to conjunctiva and ophthalmoscopy.

• Check blood pressure.

• Lab tests: Fasting blood glucose (diabetes mellitus), sickle cell prep, hemoglobin electrophoresis (sickle cell).

• Medical consultation.

Management

• No treatment recommended.

• Treat underlying medical condition.

Prognosis

Usually benign.

Dry Eye Disease (Dry Eye Syndrome, Keratoconjunctivitis Sicca)

Definition

Sporadic or chronic ocular irritation with visual disturbance due to a tear film and ocular surface abnormality.

Etiology

Any condition that causes a deficiency or imbalance in the aqueous, lipid, or mucin components of the tear film. Dry eye can be classified by mechanism (decreased tear production or increased tear evaporation), category (lid margin disease [i.e., blepharitis, meibomitis], no lid margin disease, altered tear distribution/clearance), or severity (Table 4-1). It is usually multifactorial with an inflammatory component; tear hyperosmolarity and tear film instability create a cycle of ocular surface inflammation, damage, and symptoms.

Table 4-1

Dry Eye Severity and Treatment (DEWS recommendations)

Aqueous-Deficient Dry Eye

Characterized by abnormal lacrimal gland function causing decreased tear production.

Sjögren syndrome

Dry eye, dry mouth, and arthritis with autoantibodies (to Ro (SSA) and / or La (SSB) antigens) and no connective tissue disease (primary, > 95% female) or with connective tissue disease, including rheumatoid arthritis and collagen vascular diseases (secondary).

Non-Sjögren

Hypofunction of the lacrimal gland due to other causes:

Primary lacrimal gland deficiencies

Age-related (historically labeled KCS), congenital alacrima, familial dysautonomia (Riley Day syndrome).

Secondary lacrimal gland deficiencies

Lacrimal gland infiltration, lymphoma, sarcoidosis, amyloidosis, AIDS, graft-versus-host disease, lacrimal gland ablation or denervation.

Obstruction of the lacrimal ducts

Cicatrizing conjunctivitis (Stevens–Johnson syndrome, ocular cicatricial pemphigoid, trachoma, chemical burns, radiation).

Neurosecretory block

Sensory (corneal surgery, contact lens wear, diabetes, neurotrophic keratopathy), motor (cranial nerve VII damage, systemic medications (β-blockers, antimuscarinics, antidepressants, diuretics)).

Evaporative Dry Eye

Characterized by normal lacrimal gland function but increased tear evaporation.

Intrinsic causes

Meibomian gland dysfunction (MGD)

Primary, secondary, simple, or cicatricial (see Meibomitis and Acne Rosacea sections in Chapter 3).

Lid / globe abnormalities

Conditions that result in a malposition, lagophthalmos, or proptosis with exposure of the ocular surface (see Exposure Keratopathy in Chapter 5).

Reduced blink rate

Reading/computer work (decreases blink rate by up to 60%), Parkinson’s disease.

Medications

Systemic retinoids (Accutane).

Extrinsic causes

Vitamin A deficiency

Malnutrition or malabsorption causes conjunctival xerosis and night blindness (nyctalopia) with progressive retinal degeneration; major cause of blindness worldwide.

Topical drugs

Anesthetics, preservatives (BAK – benzalkonium chloride).

Contact lens wear

Approximately 50% of contact lens wearers have dry eye symptoms.

Ocular surface disease

Cicatrizing conjunctivitis (see above), allergic conjunctivitis.

Epidemiology

Dry eye disease is estimated to affect 5–30% of the population ≥ 50 years old and is more common in women. Associated factors include age, hormone levels (menopause, androgen deficiency), environmental conditions (low humidity, wind, heat, air-conditioning, pollutants, irritants, allergens), and smoking.

Symptoms

Irritation, dryness, burning, stinging, grittiness, foreign body sensation, tearing, red eye, discharge, blurred or fluctuating vision, photophobia, contact lens intolerance, increased blinking; symptoms are variable and exacerbated by wind, smoke, and activities that reduce the blink rate (i.e., reading and computer work).

Signs

Conjunctival injection, decreased tear break-up time (< 10 seconds), decreased tear meniscus height (< 0.20 mm), excess tear film debris, corneal filaments, dry corneal surface, irregular and dull corneal light reflex, corneal and/or conjunctival staining with lissamine green, rose bengal, and fluorescein (typically in the interpalpebral space or on the inferior cornea); may have Bitot’s spot (white, foamy patch of keratinized bulbar conjunctiva [pathognomonic for vitamin A deficiency]), conjunctivochalasis (loosened bulbar conjunctiva resting on lid margin); severe cases can cause corneal ulceration, descemetocele, or perforation. May also have signs of an underlying condition (i.e., rosacea, blepharitis, lid abnormality).

Figure 4-8 Keratoconjunctivitis sicca demonstrating superficial punctate keratitis (SPK) and filaments stained with fluorescein dye.

Figure 4-9 Dry eye due to vitamin A deficiency, demonstrating diffuse staining of cornea, inferior limbus, and interpalpebral conjunctiva with rose bengal.

Figure 4-10 Dry eye demonstrating lissamine green staining of the interpalpebral conjunctiva.

Differential Diagnosis

See above; also allergic conjunctivitis, medicamentosa, contact lens overwear, trichiasis.

Evaluation

• Complete history with attention to severity, duration, exacerbating factors, prior treatment, contact lens wear, previous eye/eyelid surgery, systemic diseases and medications. Consider using a questionnaire such as the Ocular Surface Disease Index (OSDI), Dry Eye Questionnaire (DEQ), National Eye Institute Visual Function Questionnaire (NEI VFQ-25), or McMonnies questionnaire.

• Complete eye exam with attention to lids, tear film (break-up time and height of meniscus), conjunctiva, and cornea (staining with lissamine green or rose bengal [dead and degenerated epithelial cells], and/or fluorescein [only dead epithelial cells]).

• Schirmer’s test: Two tests exist, but they are usually not performed as originally described. One is done without and one is done with topical anesthesia. The inferior fornix is dried with a cotton-tipped applicator, and a strip of standardized filter paper (Whatman #41, 5 mm width) is placed in each lower lid at the junction of the lateral and middle thirds. After 5 minutes, the strips are removed and the amount of wetting is measured. Normal results are 15 mm or greater without anesthesia (basal + reflex tearing), and 10 mm or greater with topical anesthesia (basal tearing). Schirmer’s test often produces variable results so its usefulness may be limited.

• Alternatively, consider the phenol red thread test: Similar to the Schirmer’s test but uses a special cotton thread that changes color as tears are absorbed for 30 seconds; less irritating and may be more reproducible.

• Lab tests: Consider tear lactoferrin and lysozyme (decreased), matrix metalloproteinase 9 (MMP-9) (> 40 ng/mL), tear osmolarity (> 316 mOsm / L), and impression cytology (detects reduced goblet cell density and squamous metaplasia; rarely used clinically).

• Consider corneal topography (computerized videokeratography): Irregular or broken mires with resulting abnormal topography and blank areas due to poor-quality tear film and dry spots. Surface regularity index (SRI) value correlates with severity of dry eye.

• Electroretinogram (reduced), electro-oculogram (abnormal), and dark adaptation (prolonged) in vitamin A deficiency.

• Consider medical consultation for systemic diseases.

Management

• Reduce/eliminate associated factors.

• The mainstay of therapy is topical lubrication with artificial tears up to q1h and gel or ointment qhs. Preservative-free tear formulations should be used if the frequency of administration is greater than qid.

• Additional treatment is performed in a stepwise fashion and includes: hydroxypropyl methylcellulose (Lacrisert), topical cyclosporine 0.05% (Restasis) bid for at least 3 months, short course (1–2 weeks) of topical steroid (Lotemax, Alrex, Pred Mild, or FML) bid to qid, punctal occlusion (with plugs or cautery), nutritional supplements (i.e., oral flaxseed / fish oils [omega-3 fatty acids]), bandage contact lens, moisture chamber goggles, lid taping at bedtime, and tarsorrhaphy for more severe cases. Recommendations of the 2007 Dry Eye Workshop (DEWS) are based on severity (1–4) of symptoms (discomfort, fatigue, visual disturbance) and signs (lid, tear film, conjunctiva, cornea) (see Table 4-1).

• Consider acetylcysteine 10% (Mucomyst) qd to qid for mucus strands or filaments.

• Consider autologous serum drops or Boston ocular surface prosthesis (PROSE lens) in severe cases.

• Consider oral cholinergic agonists (pilocarpine, cevimeline) to increase tear production, especially in patients with Sjögren syndrome.

• Treat underlying condition:

• Acne rosacea/blepharitis: see Chapter 3.

• Vitamin A deficiency: Vitamin A replacement (vitamin A 15,000 IU po qd).

• Androgen deficiency: Consider treatment with transdermal testosterone cream (3%) to closed eyelids, and/or topical DHEA (compounded eye drops).

• Lid malposition: Consider surgical repair.

Prognosis

Depends on underlying condition; severe cases may be difficult to manage.

Inflammation

Definition

Chemosis

Edema of conjunctiva; may be mild with boggy appearance or massive with tense ballooning.

Figure 4-11 Chemosis with extensive ballooning of conjunctiva and prolapse over lower lid nasally. Temporally, the edges of the elevated conjunctiva are delineated by the light reflexes from the tear film.

Follicles

Small, translucent, avascular mounds of plasma cells and lymphocytes found in epidemic keratoconjunctivitis (EKC), herpes simplex virus, chlamydia, molluscum, or drug reactions.

Figure 4-12 Follicular conjunctivitis demonstrating inferior palpebral follicles with the typical gelatinous bump appearance.

Figure 4-13 Large, gelatinous, tarsal follicles in a patient with acute trachoma.

Granuloma

Collection of giant multinucleated cells found in chronic inflammation from sarcoid, foreign body, or chalazion.

Hyperemia

Redness and injection of conjunctiva.

Figure 4-14 Hyperemia. The dilated conjunctival vessels produce a diffuse redness (injection).

Membranes

A true membrane is a firmly adherent, fibrinous exudate that bleeds and scars when removed; found in bacterial conjunctivitis (Streptococcus species, Neisseria gonorrhoeae, Corynebacterium diphtheriae), Stevens–Johnson syndrome, and burns. A pseudomembrane is a loosely attached, avascular, fibrinous exudate found in EKC and mild allergic or bacterial conjunctivitis.

Figure 4-15 Pseudomembrane evident as a thick yellow coating in a patient with epidemic keratoconjunctivitis.

Papillae

Vascular reaction consisting of fibrovascular mounds with central vascular tuft; nonspecific finding in any conjunctival irritation or conjunctivitis; can be large (“cobblestones” or giant papillae).

Figure 4-16 Large papillae in a patient with vernal keratoconjunctivitis. The central vascular cores are clearly visible as dots within the papillae.

Phlyctenule

Focal, nodular, vascularized infiltrate of polymorphonuclear leukocytes and lymphocytes with central necrosis due to hypersensitivity to Staphylococcus species, Mycobacterium species, Candida species, Coccidioides, Chlamydia, or nematodes; located on the bulbar conjunctiva or at the limbus; can march across the cornea, causing vascularization and scarring behind the leading edge.

Figure 4-17 Phlyctenule creeping across the cornea is demonstrated by the white infiltrate with trailing neovascularization.

Symptoms

Red eye, swelling, itching, foreign body sensation; may have discharge, photophobia, and tearing.

Signs

See above; depends on type of inflammation.

Differential Diagnosis

Any irritation of conjunctiva (allergic, infectious, autoimmune, chemical, foreign body, idiopathic).

Evaluation

• Complete ophthalmic history and eye exam with attention to preauricular lymphadenopathy, everting lids, conjunctiva, cornea, and characteristics of discharge if present.

• Lab tests: Cultures and smears of conjunctiva, cornea, and discharge for infectious causes.

Management

• Treatment depends on etiology; usually supportive.

• Topical vasoconstrictor, nonsteroidal anti-inflammatory drug (NSAID), antihistamine, mast cell stabilizer, or mast cell stabilizer and antihistamine combination (see Table 4-2); severe cases may require topical steroid (prednisolone acetate 1% qid) or topical antibiotic (bacitracin-polymixin B sulfate [Polysporin] drops or erythromycin ointment qid or both).

Table 4-2

Topical Medications Available for Management of Allergic Conjunctivitis

• Membranes and pseudomembranes may require debridement.

• Discontinue offending agent if due to allergic reaction.

Prognosis

Depends on etiology; most are benign and self-limited (see Conjunctivitis section below).

Conjunctivitis

Definition

Infectious or noninfectious inflammation of the conjunctiva classified as acute (shorter than 4 weeks’ duration) or chronic (longer than 4 weeks’ duration).

Acute Conjunctivitis

Infectious

Gonococcal

Hyperacute presentation with severe purulent discharge, chemosis, papillary reaction, preauricular lymphadenopathy, and lid swelling. N. gonorrhoeae can invade intact corneal epithelium and cause infectious keratitis (see Chapter 5).

Nongonococcal bacterial

Seventy percent due to Gram-positive organisms, 30% due to Gram-negative organisms; usually caused by Staphylococcus aureus, Streptococcus pneumoniae, Haemophilus species, or Moraxella catarrhalis. Spectrum of clinical presentations ranging from mild (signs include minimal lid edema, scant purulent discharge) to moderate (significant conjunctival injection, membranes); usually no preauricular lymphadenopathy or corneal involvement. Most common cause of acute conjunctivitis in children under 3 years old (50–80%).

Figure 4-18 Bacterial conjunctivitis with mucopurulent discharge adherent to the upper tarsal conjunctiva.

Adenoviral

Most common cause of viral conjunctivitis (“pink eye”); signs include lid edema, serous discharge, pseudomembranes; may have preauricular lymphadenopathy and corneal subepithelial infiltrates. Transmitted by contact and contagious for 12–14 days; 51 serotypes, 1 / 3 associated with eye infection:

Epidemic keratoconjunctivitis (EKC)

Caused by types 8, 19, and 37.

Pharyngoconjunctival fever

Caused by types 3, 4, 5, and 7.

Nonspecific follicular conjunctivitis

Caused by types 1–11 and 19.

Figure 4-19 Adenoviral conjunctivitis due to epidemic keratoconjunctivitis with characteristic subepithelial infiltrates.

Figure 4-20 Herpes simplex viral conjunctivitis with characteristic lid vesicles.

Herpes simplex virus

Primary disease in children causes lid vesicles; may also have fever, preauricular lymphadenopathy, and an upper respiratory infection.

Herpes zoster virus

Herpes zoster ophthalmicus can cause conjunctival injection, hemorrhages, vesicles, papillary or follicular reaction, membrane or pseudomembrane; may develop ulceration, scarring, and symblepharon; may have other ocular complications (see Chapters 3 and 5).

Pediculosis

Results from contact with pubic lice (sexually transmitted); may be unilateral or bilateral (see Chapter 3).

Allergic

Seasonal

Most common (50% of allergic conjunctivitis); occurs in individuals of all ages; associated with hay fever, airborne allergens (i.e., pollen).

Atopic keratoconjunctivitis

Rare (3% of allergic conjunctivitis); occurs in adults; not seasonal; associated with atopy (rhinitis, asthma, dermatitis). Similar features as vernal keratoconjunctivitis but papillae usually smaller and conjunctiva has milky edema; also thickened and erythematous lids, corneal neovascularization, cataracts (10%), and keratoconus.

Figure 4-21 Atopic keratoconjunctivitis demonstrating corneal vascularization and scarring with symblepharon.

Vernal keratoconjunctivitis

Very rare (< 1% of allergic conjunctivitis); occurs in children; seasonal (warm months); male predilection; lasts 5–10 years, then resolves. Associated with family history of atopy; signs include intense itching, ropy discharge, giant papillae (cobblestones), Horner–Trantas dots (collections of eosinophils at limbus), shield ulcer, and keratitis (50%). Histologically, more than two eosinophils per high-power field is pathognomonic.

Figure 4-22 Vernal keratoconjunctivitis demonstrating “cobblestones” (giant papillae).

Figure 4-23 Horner–Trantas dot appears as an elevated white bump at the limbus.

Toxic

Follicular reaction due to eye drops (especially neomycin, aminoglycoside antibiotics, antiviral medications, atropine, miotic agents, brimonidine [Alphagan], apraclonidine [Iopidine], epinephrine, and preservatives including contact lens solutions [i.e., thimerosal]).

Chronic Conjunctivitis

Infectious

Chlamydial

Trachoma

Leading cause of blindness worldwide; caused by serotypes A–C. Signs include follicles, Herbert’s pits (scarred limbal follicles), superior pannus, superficial punctate keratitis, upper tarsal scarring (Arlt’s line).

Figure 4-24 Trachoma demonstrating linear pattern of upper tarsal scarring. Also note the abundant concretions that appear as yellow granular aggregates.

Figure 4-25 Chlamydial conjunctivitis with follicles in the inferior fornix.

Inclusion conjunctivitis

Caused by serotypes D–K. Signs include chronic, follicular conjunctivitis, subepithelial infiltrates, and no membrane; associated with urethritis in 5%.

Lymphogranuloma venereum

Caused by serotype L. Associated with Parinaud’s oculoglandular syndrome (see below), conjunctival granulomas, and interstitial keratitis.

Molluscum contagiosum

Usually appears with one or more, umbilicated, shiny papules on the eyelid. Associated with follicular conjunctivitis; with multiple lesions, consider human immunodeficiency virus (HIV) (see Chapter 3).

Allergic

Perennial

Occurs in individuals of all ages. Associated with animal dander, dust mites.

Giant papillary conjunctivitis

Occurs in contact lens wearers (> 95% of giant papillary conjunctivitis cases); also secondary to prosthesis, foreign body, or exposed suture. Signs include itching, ropy discharge, blurry vision, and pain/irritation with contact lens wear.

Figure 4-26 Giant papillary conjunctivitis demonstrating large papillae in upper tarsal conjunctiva.

Toxic

Follicular reaction due to eye drops or contact lens solutions (see above).

Other

Superior limbic keratoconjunctivitis

Occurs in middle-aged females; 50% have thyroid disease; usually bilateral and asymmetric; may be secondary to contact lens use (see Chapter 5). Signs include boggy edema, redundancy and injection of superior conjunctiva, superficial punctate keratitis, filaments, and no discharge; symptoms are worse than signs.

Figure 4-27 Superior limbic keratoconjunctivitis demonstrating the typical staining of the central superior conjunctiva with rose bengal dye.

Kawasaki’s disease

Mucocutaneous lymph node syndrome of unknown etiology; occurs in children under 5 years old; more common in Japanese. Diagnosis based on 5 of 6 criteria: (1) fever (≥ 5 days), (2) bilateral conjunctivitis, (3) oral mucosal changes (erythema, fissures, “strawberry tongue”), (4) rash, (5) cervical lymphadenopathy, and (6) peripheral extremity changes (edema, erythema, desquamation). Associated with polyarteritis especially of coronary arteries; may be fatal (1–2%).

Ligneous

Rare, idiopathic, bilateral, membranous conjunctivitis; occurs in children. A thick, white, woody infiltrate and plaque develops on the upper tarsal conjunctiva.

Figure 4-28 Ligneous conjunctivitis demonstrating thick, yellow-white plaque on superior tarsal conjunctiva.

Parinaud’s oculoglandular syndrome

Unilateral conjunctivitis with conjunctival granulomas and preauricular/submandibular lymphadenopathy; may have fever, malaise, and rash. Due to cat-scratch fever, tularemia, sporotrichosis, tuberculosis, syphilis, lymphogranuloma venereum, Epstein–Barr virus, mumps, fungi, malignancy, or sarcoidosis.

Figure 4-29 Parinaud’s oculoglandular syndrome. There is marked eyelid swelling and erythema in this patient with an affected left eye.

Ophthalmia neonatorum

Occurs in newborns; may be toxic (silver nitrate) or infectious (bacteria [especially N. gonorrhoeae], herpes simplex virus, chlamydia [may have otitis and pneumonitis]).

Figure 4-30 Hyperacute gonorrheal conjunctivitis demonstrating conjunctival injection and purulent discharge in a newborn.

Symptoms

Red eye, swelling, itching, burning, foreign body sensation, tearing, discharge, crusting of lashes; may have photophobia and decreased vision.

Signs

Normal or decreased visual acuity, lid edema, conjunctival injection, chemosis, papillae, follicles, membranes, petechial hemorrhages, concretions, discharge; may have preauricular lymphadenopathy, tarsal conjunctival filament, subepithelial corneal infiltrates, punctate corneal staining, corneal ulcers, and cataract.

Differential Diagnosis

See above; also blepharitis (may present as “recurrent conjunctivitis”), medicamentosa (toxic reaction commonly associated with preservatives [i.e., benzalkonium chloride (BAK)] in medications, as well as antiviral medications, antibiotics, miotic agents, dipivefrin [Propine], apraclonidine [Iopidine], and atropine), dacryocystitis, nasolacrimal duct obstruction.

Evaluation

• Complete ophthalmic history and eye exam with attention to preauricular lymphadenopathy, everting lids, characteristics of discharge, conjunctiva, cornea, and anterior chamber.

• Lab tests: Consider cultures and smears of conjunctiva and cornea (mandatory for suspected bacterial cases). Consider RPS Adeno Detector test (for suspected viral cases).

• Consider pediatric consultation.

Management

• Treatment depends on etiology; usually supportive with medications, compresses, and debridement of membranes for symptomatic relief.

BACTERIAL

• Topical broad-spectrum antibiotic (gatifloxacin [Zymaxid] qid, moxifloxacin [Vigamox, Moxeza] tid, besifloxacin [Besivance] tid, or azithromycin [Azasite] bid/qd).

• May require systemic antibiotics especially in children.

• Remove discharge with irrigation and membranes with sterile cotton-tipped applicator.

VIRAL

• Topical lubrication with artificial tears and topical vasoconstrictor, NSAID, antihistamine, mast cell stabilizer, or mast cell stabilizer and antihistamine combination (see Table 4-2).

• Topical antibiotic (polymyxin B sulfate-trimethoprim [Polytrim] drops qid, erythromycin or bacitracin ointment qid to tid) for corneal epithelial defects.

• Topical steroid (fluorometholone alcohol 0.1% [FML] qid) for subepithelial infiltrates in EKC.

• Topical antiviral (ganciclovir gel 0.15% [Zirgan] or trifluridine 1% [Viroptic] 5 times/day) for herpes simplex.

• Topical antibiotic (polymyxin B sulfate-trimethoprim [Polytrim] drops qid, erythromycin or bacitracin ointment tid) for herpes zoster; consider topical steroid (prednisolone acetate 1% qid) if severe.

OTHER INFECTIONS

• N. gonorrhoeae, Chlamydia, and Parinaud’s oculoglandular syndrome (e.g., cat-scratch disease, tularemia, syphilis, tuberculosis, etc.) require systemic antibiotics based on causative organism.

ALLERGIC

• Identify and avoid/eliminate inciting agent(s); may require allergic patch testing to determine causative allergens.

• Topical lubrication with artificial tears and topical vasoconstrictor, NSAID, antihistamine, mast cell stabilizer, or mast cell stabilizer and antihistamine combination (see Table 4-2).

• Consider mild topical steroid especially for severe allergic keratoconjunctivitis; start with fluorometholone alcohol 0.1% (FML) qid or loteprednol etabonate 0.2% (Alrex) qid, change to loteprednol etabonate 0.5% (Lotemax) or prednisolone 1% qid to q1h in severe cases. Solution (phosphate [Inflamase Mild, AK-Pred]) works better than suspension (acetate [Pred Mild, Econopred]).

• Consider systemic antihistamine (diphenhydramine [Benadryl] 25–50 mg po q6h prn).

GIANT PAPILLARY CONJUNCTIVITIS

• Clean, change, or discontinue use of contact lenses; change to preservative-free contact lens cleaning solution (see Chapter 5).

SUPERIOR LIMBIC KERATOCONJUNCTIVITIS

• Silver nitrate solution, bandage contact lens, conjunctival cautery, or conjunctival recession and resection.

• Steroids do not help.

• Cromolyn sodium 4% (Crolom) qid or olopatadine hydrochloride 0.1% (Patanol) bid may be useful (see Chapter 5).

ATOPIC AND VERNAL KERATOCONJUNCTIVITIS

• Consider topical cyclosporine (1–2%) qid.

• Consider supratarsal steroid injection (0.25–0.50 mL dexamethasone or triamcinolone acetate [Kenalog]).

KAWASAKI’S DISEASE

• Pediatric consultation and hospital admission.

• Systemic steroids are contraindicated.

LIGNEOUS

• May respond to topical steroids, mucolytics, or cyclosporine.

Prognosis

Usually good; subepithelial infiltrates in adenoviral conjunctivitis cause variable decreased vision for months.

Degenerations

Definition

Secondary degenerative changes of the conjunctiva.

Amyloidosis

Yellow-white or salmon-colored, avascular deposits; may be due to primary (localized) or secondary (systemic) amyloidosis.

Figure 4-31 Yellowish avascular deposits in inferior conjunctiva from amyloidosis.

Concretions

Yellow-white inclusion cysts filled with keratin and epithelial debris in fornix or palpebral conjunctiva; associated with aging and chronic conjunctivitis; can erode through overlying conjunctiva causing foreign body sensation (see Figure 4-24).

Pinguecula

Yellow-white, subepithelial lesion of abnormal collagen at the medial or temporal limbus; due to actinic changes; elastotic degeneration seen histologically; may become inflamed (pingueculitis); may calcify over time.

Figure 4-32 Pinguecula appears as a yellow-white elevated mass near the medial limbus.

Pterygium

Triangular fibrovascular tissue in interpalpebral space involving cornea; often preceded by pinguecula; destroys Bowman’s membrane; may have iron line at leading edge (Stocker’s line); induces astigmatism and may cause decreased vision.

Figure 4-33 Large nasal and smaller temporal pterygia. Note the typical triangular, wedge-shaped configuration, and also the white corneal scarring at the leading edges.

Symptoms

Asymptomatic; may have red eye, foreign body sensation, decreased vision; may notice bump or growth; may have contact lens intolerance.

Signs

See above.

Differential Diagnosis

Cyst, squamous cell carcinoma, conjunctival intraepithelial neoplasia, episcleritis, scleritis, phlyctenule.

Evaluation

• Complete ophthalmic history and eye exam with attention to conjunctiva and cornea.

Management

• Often no treatment necessary.

• Topical lubrication with artificial tears up to q1h.

• Consider limited use of vasoconstrictor (naphazoline [Naphcon] qid) for inflamed pinguecula or pterygium.

• Consider short course (1–2 weeks) of topical steroid (Alrex or FML) bid to qid for pingueculitis.

• Surgical excision of pterygium for chronic inflammation, cosmesis, contact lens intolerance, or involvement of visual axis.

• Concretions are easily unroofed and removed with a 27–30-gauge ½-inch needle if symptomatic.

Prognosis

Good; about one-third of pterygia recur after simple excision, recurrences are reduced by conjunctival autograft, β-irradiation, thiotepa, or mitomycin C.

Ocular Cicatricial Pemphigoid

Definition

Systemic vesiculobullous disease of mucous membranes resulting in bilateral, chronic, cicatrizing conjunctivitis; other mucous membranes frequently involved, including oral (up to 90%), esophageal, tracheal, and genital; skin involved in up to 30% of cases.

Etiology

Usually idiopathic (probably autoimmune mechanism) or drug induced (may occur with epinephrine, timolol, pilocarpine, echothiophate iodide [Phospholine Iodide], or idoxuridine).

Epidemiology

Incidence of 1 in 20,000; usually occurs in females (2 : 1) over 60 years old; associated with HLA-DR4, DQw3.

Symptoms

Red eye, dryness, foreign body sensation, tearing, decreased vision; may have dysphagia or difficulty in breathing.

Signs

Normal or decreased visual acuity, conjunctival injection and scarring, dry eye, symblepharon (fusion or attachment of eyelid to bulbar conjunctiva), ankyloblepharon (fusion or attachment of upper and lower eyelids), foreshortened fornices, trichiasis, entropion, keratitis, corneal ulcer, scarring, and vascularization, conjunctival and corneal keratinization, and oral lesions; corneal perforation and endophthalmitis can occur.

Figure 4-34 Ocular cicatricial pemphigoid demonstrating symblepharon and foreshortening of the inferior fornix.

Figure 4-35 Ocular cicatricial pemphigoid demonstrating advanced stage with corneal vascularization and symblepharon.

Figure 4-36 Keratoprosthesis in eye with advanced ocular cicatricial pemphigoid.

Differential Diagnosis

Stevens–Johnson syndrome, chemical burn, squamous cell carcinoma, scleroderma, infectious or allergic conjunctivitis, trachoma, sarcoidosis, ocular rosacea, radiation, linear immunoglobulin A dermatosis, practolol-induced conjunctivitis.

Evaluation

• Complete ophthalmic history and eye exam with attention to lids, conjunctiva, and cornea.

• Conjunctival biopsy (immunoglobulin and complement deposition in basement membrane).

Management

• Topical lubrication with preservative-free artificial tears up to q1h and ointment qhs.

• Topical antibiotic (polymyxin B sulfate-trimethoprim [Polytrim] drops qid or erythromycin ointment tid) for corneal epithelial defects.

• Consider punctal occlusion, tarsorrhaphy, or Boston ocular surface prosthesis (PROSE lens).

• Often requires treatment with systemic steroids or immunosuppressive agents (dapsone [contraindicated in patients with glucose 6-phosphate dehydrogenase deficiency], cyclophosphamide); should be performed by a cornea or uveitis specialist.

• Consider surgery for entropion, trichiasis, symblepharon, ankyloblepharon, or corneal scarring; may require mucous membrane grafting; keratoprosthesis in advanced cases.

Prognosis

Poor; chronic progressive disease with remissions and exacerbations, surgery often initiates exacerbations.

Stevens–Johnson Syndrome (Erythema Multiforme Major)

Definition

Acute, usually self-limited (up to 6 weeks), cutaneous, bullous disease with mucosal ulceration resulting in acute membranous conjunctivitis.

Etiology

Usually drug-induced (may occur with sulfonamides, penicillin, aspirin, barbiturates, isoniazid, or phenytoin [Dilantin]) or infectious (herpes simplex virus, Mycoplasma species, adenovirus, Streptococcus species).

Symptoms

Fever, upper respiratory infection, headache, malaise, skin eruption, decreased vision, pain, red eye, swelling, and oral mucosal ulceration.

Signs

Fever, skin eruption (target lesions), mucous membrane ulceration and crusting, decreased visual acuity, conjunctival injection, discharge, membranes, dry eye, symblepharon (fusion or attachment of eyelid to bulbar conjunctiva), trichiasis, keratitis, corneal ulcer, scarring, vascularization, and keratinization.

Differential Diagnosis

Ocular cicatricial pemphigoid, chemical burn, squamous cell carcinoma, scleroderma, infectious or allergic conjunctivitis, trachoma, sarcoidosis, ocular rosacea, radiation.

Evaluation

• Complete ophthalmic history and eye exam with attention to systemic mucous membranes, lids, conjunctiva, and cornea.

• Medical consultation.

Figure 4-37 Stevens–Johnson syndrome demonstrating tarsal scarring.

Figure 4-38 Stevens–Johnson syndrome demonstrating keratinization of the ocular surface with a dry, wrinkled appearance. Note the resulting irregular, diffuse corneal reflex.

Management

• Supportive, topical lubrication with preservative-free artificial tears up to q1h and ointment qhs.

• Topical antibiotic (polymyxin B sulfate-trimethoprim [Polytrim] drops qid or erythromycin ointment tid) for corneal epithelial defects.

• Consider topical steroid (prednisolone acetate 1% up to q2h) depending on severity of inflammation.

• Consider oral steroids (prednisone 60–100 mg po qd) in very severe cases; check purified protein derivative (PPD) and controls, blood glucose, and chest radiographs before starting systemic steroids.

• Add H₂-blocker (ranitidine [Zantac] 150 mg po bid) or proton pump inhibitor when administering systemic steroids.

• May require punctal occlusion, tarsorrhaphy, or Boston ocular surface prosthesis (PROSE lens) for more severe cases.

• Consider surgery for trichiasis, symblepharon, or corneal scarring.

Prognosis

Fair; not progressive (in contrast to ocular cicatricial pemphigoid), recurrences are rare, but up to 30% mortality.

Tumors

Congenital

Hamartoma

Derived from abnormal rest of cells, composed of tissues normally found at same location (e.g., telangiectasia, lymphangioma).

Choristoma

Derived from abnormal rest of cells, composed of tissues not normally found at that location.

Dermoid

White-yellow, solid, round, elevated nodule, often with visible hairs on surface and lipid deposition anterior to its corneal edge; composed of dense connective tissue with pilosebaceous units and stratified squamous epithelium; usually located at inferotemporal limbus; may be part of Goldenhar’s syndrome with dermoids, preauricular skin tags, and vertebral anomalies (see Chapter 5).

Dermolipoma

Similar appearance to dermoid but composed of adipose tissue with keratinized surface; usually located superotemporally extending into orbit.

Epibulbar osseous choristoma

Solitary, white nodule composed of compact bone that develops from episclera; freely moveable; usually located superotemporally.

Epithelial

Cysts

Fluid-filled cavity within the conjunctiva, defined by its lining (ductal or inclusion). Often due to trauma or inflammation, can be congenital.

• No treatment necessary.

• Consider excision; must remove inner lining to prevent recurrence.

Figure 4-39 Conjunctival inclusion cyst appears as a clear elevation over which the slit-beam bends.

Papilloma

Red, gelatinous lesions composed of proliferative epithelium with fibrovascular cores; may be pedunculated or sessile, solitary or multiple. Often associated with human papillomavirus.

• May resolve spontaneously.

• Consider excision with or without cryotherapy, topical interferon α-2b, or topical mitomycin C.

• Consider oral cimetidine or carbon dioxide laser treatment for recalcitrant cases.

Figure 4-40 Papilloma demonstrating the typical elevated appearance with central vascular fronds.

Figure 4-41 Squamous papilloma with elevated, gelatinous appearance.

Conjunctival Intraepithelial Neoplasia

White, gelatinous, conjunctival dysplasia confined to the epithelium; usually begins at limbus and is a precursor of squamous cell carcinoma.

• Excisional biopsy; consider topical 5-fluorouracil, topical mitomycin C, or interferon α-2b (topical or subconjunctival).

Figure 4-42 Conjunctival intraepithelial neoplasia demonstrating pink, nodular, gelatinous, vascularized appearance at the limbus.

Squamous Cell Carcinoma

Interpalpebral, exophytic, gelatinous, papillary appearance with loops of abnormal vessels; may have superficial invasion and extend onto cornea. Most common conjunctival malignancy in the US; more common in elderly, Caucasians (90%), and males (81%); associated with ultraviolet radiation, human papillomavirus, and heavy smoking; suspect AIDS in patients < 50 years of age. Intraocular invasion (2–8%), orbital invasion (12–16%), and metastasis are rare; recurrence after excision is < 10%; mortality rate of up to 8%.

• MRI to rule out orbital involvement if suspected.

• Excisional biopsy with episclerectomy, corneal epitheliectomy with 100% alcohol, and cryotherapy to bed of lesion; should be performed by a cornea or tumor specialist.

• Consider topical 5-fluorouracil, topical mitomycin C, or interferon α-2b (topical or subconjunctival) especially for recurrence.

• Exenteration with adjunctive radiation therapy for orbital involvement.

• Medical consultation and systemic workup.

Figure 4-43 Squamous cell carcinoma with gelatinous growth and abnormal vascular loops.

Melanocytic

Nevus

Mobile, discrete, elevated, variably pigmented lesion that contains cysts; may be junctional, subepithelial, or compound. May enlarge during puberty; rarely becomes malignant.

• Observation; consider serial photographs and clinical examination for any growth that would be suspicious for malignant melanoma.

Figure 4-44 Elevated (A) pigmented and (B) nonpigmented conjunctival nevus.

Ocular Melanocytosis

Unilateral, increased uveal, scleral, and episcleral pigmentation appearing as blue-gray patches. More common in Caucasians.

• No treatment necessary.

Figure 4-45 Ocular melanocytosis demonstrating blue-gray, patchy pigmentation.

Oculodermal Melanocytosis (Nevus of Ota)

Increased uveal, scleral, episcleral, and periorbital skin pigmentation; more common in Asians and African-Americans; increased risk of congenital glaucoma; uveal melanoma may rarely develop in Caucasians (see Chapter 10).

• Periodic examinations to monitor for evidence of malignant transformation.

Figure 4-46 Oculodermal melanocytosis (nevus of Ota) of the left eye. Note the prominent scleral pigmentation; the periorbital skin changes are difficult to see in this photo.

Primary Acquired Melanosis

Mobile, patchy, diffuse, flat, brown lesions without cysts; indistinct margins, may grow and involve the cornea. Occurs in middle-aged Caucasian adults; accounts for 11% of conjunctival tumors and 21% of pigmented lesions. Histologically may have atypia; 13% with severe atypia progress to malignant melanoma.

• Excisional biopsy with cryotherapy.

• Consider topical interferon α-2b or mitomycin C for recurrence.

Figure 4-47 Primary acquired melanosis demonstrating mottled, brown, patchy pigmentation.

Secondary Acquired Melanosis

Hyperpigmentation due to racial variations, actinic stimulation, radiation, pregnancy, Addison’s disease, or inflammation; may also occur with topical prostaglandin analogues; usually perilimbal.

• No treatment necessary.

Figure 4-48 Secondary acquired melanosis (racial pigmentation) demonstrating typical perilimbal, brown, homogeneous pigmentation.

Malignant Melanoma

Nodular, pigmented lesion containing vessels, but no cysts; may arise from primary acquired melanosis (70%), nevus (20%), or de novo (10%). Mainly occurs in middle-aged Caucasian adults; accounts for 2% of ocular malignancies. Twenty-five percent risk of metastasis (to parotid and submandibular nodes first); 25–45% mortality.

• Consider ultrasound biomicroscopy (UBM) to rule out extrascleral extension of ciliary body melanoma.

• Excisional biopsy using “no touch” technique with episclerectomy, corneal epitheliectomy with 100% alcohol, and cryotherapy (double freeze–thaw); consider topical mitomycin C; should be performed by a cornea or tumor specialist.

• May require exenteration, but does not improve prognosis.

• Medical consultation and systemic workup.

Figure 4-49 Malignant melanoma with nodular, pigmented, vascular lesion at the limbus.

Figure 4-50 Malignant melanoma demonstrating irregular pigmented growth at limbus and onto cornea with vascularization.

Stromal

Cavernous Hemangioma

Red patch on conjunctiva; may bleed and be associated with other ocular hemangiomas or systemic disease.

• No treatment necessary.

Figure 4-51 Cavernous hemangioma demonstrating red patch of dilated blood vessels in the inferior fornix.

Juvenile Xanthogranuloma

Yellow-orange nodules composed of vascularized, lipid-containing histiocytes; can also involve iris and skin; often regresses spontaneously.

• No treatment necessary.

Kaposi’s Sarcoma

Single or multiple, flat or elevated, deep red to purple plaques (malignant granulation tissue); may cause recurrent subconjunctival hemorrhage; may involve orbit; also found on skin. Occurs in immunocompromised individuals, especially HIV-positive patients.

• Consider excision, cryotherapy, chemotherapy, or radiation therapy; should be performed by a cornea or tumor specialist.

• Medical consultation and systemic workup.

Figure 4-52 Kaposi’s sarcoma demonstrating beefy, red, large, nodular mass in the inferior fornix.

Lymphangiectasis

Cluster of elevated clear cysts that represent dilated lymphatics; may have areas of hemorrhage.

• Consider excision.

Figure 4-53 Lymphangiectasis demonstrating cluster of cysts medially near the caruncle.

Figure 4-54 Lymphangiectasis with clear cysts.

Lymphoid

Figure 4-55 Malignant lymphoma with salmon-colored lesion.

Figure 4-56 Lymphoid tumor demonstrating typical salmon-patch appearance.

Single or multiple, smooth, flat, salmon-colored patches. Usually occurs in middle-aged adults. Spectrum of disease from benign reactive lymphoid hyperplasia to malignant lymphoma (non-Hodgkin’s; less aggressive MALT [mucosa-associated lymphoid tissue] or more malignant non-MALT); may involve orbit, may develop systemic lymphoma (see Chapter 1).

• Tissue biopsy with immunohistochemical studies on fresh specimens required for diagnosis; should be performed by a cornea or tumor specialist.

• External beam radiation for lesion limited to conjunctiva.

• May require systemic chemotherapy.

• Medical and oncology consultations and systemic workup.

Figure 4-57 Lymphoma involving the entire conjunctiva.

Pyogenic Granuloma

Red, fleshy, polypoid mass at the site of chronic inflammation; often follows surgical or accidental trauma. Misnomer, because it is neither pyogenic nor a granuloma, but rather granulation tissue.

• Topical steroid.

• Consider excision.

Figure 4-58 Pyogenic granuloma appearing as a large, fleshy, vascular, pedunculated growth.

Caruncle

Tumors that affect the conjunctiva may also occur in the caruncle, including (in order of frequency): papilloma, nevus, inclusion cyst, malignant melanoma, also sebaceous cell carcinoma, and oncocytoma (oxyphilic adenoma; fleshy, yellow-tan cystic mass from transformation of epithelial cells of accessory lacrimal glands; slowly progressive).

• Consider excisional biopsy.

• Medical consultation and systemic workup for malignant lesions.

Figure 4-59 Nevus of the caruncle appearing as a brown pigmented spot.

Figure 4-60 Papilloma of the caruncle appearing as a large, vascular, pedunculated mass.

Episcleritis

Definition

Sectoral (70%) or diffuse (30%) inflammation of episclera.

Epidemiology

Eighty percent simple; 20% nodular; 33% bilateral.

Etiology

Idiopathic, tuberculosis, syphilis, herpes zoster, rheumatoid arthritis, other collagen vascular diseases.

Symptoms

Red eye; may have mild pain.

Signs

Subconjunctival and conjunctival injection, usually sectoral; may have chemosis, episcleral nodules, anterior chamber cells and flare.

Figure 4-61 Episcleritis demonstrating characteristic sectoral injection.

Differential Diagnosis

Scleritis, iritis, pingueculitis, myositis, phlyctenule, staphylococcal marginal keratitis, superior limbic keratoconjunctivitis.

Evaluation

• Complete ophthalmic history and eye exam with attention to pattern of conjunctival and scleral injection and blanching with topical phenylephrine (scleritis is painful, has violaceous hue, and does not blanch with topical vasoconstrictor [phenylephrine 2.5%]), cornea, and anterior chamber.

• Consider scleritis workup (see below) in recurrent or bilateral cases.

Management

• Consider limited use of vasoconstrictor (naphazoline [Naphcon] qid) for mild cases.

• May require topical steroid (fluorometholone acetate [Flarex] qid) or oral NSAID (indometacin 50 mg po qd to bid) for severe cases.

Prognosis

Good; usually self-limited; may be recurrent in 67% of cases.

Scleritis

Definition

Inflammation of sclera, can be anterior (98%) or posterior (2%) (see Chapter 10).

Epidemiology

Anterior form may be diffuse (40%), nodular (44%), or necrotizing (14%) with or without (scleromalacia perforans) inflammation; more than 50% are bilateral; associated systemic disease in 50% of cases.

Etiology

Collagen vascular disease in 30% of cases (most commonly rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus, polyarteritis nodosa, Wegener’s granulomatosis, and relapsing polychondritis); also herpes zoster, syphilis, tuberculosis, leprosy, gout, porphyria, postsurgical, and idiopathic.

Symptoms

Pain, photophobia, swelling, red eye, decreased vision (except scleromalacia).

Signs

Normal or decreased visual acuity, subconjunctival and conjunctival injection with violaceous hue, chemosis, scleral edema, scleral nodule(s), globe tenderness to palpation; may have anterior chamber cells and flare (30%), corneal infiltrate or thinning, scleral thinning (30%); posterior type may have chorioretinal folds, focal serous retinal detachments, vitritis, and optic disc edema (see Figures 10-170 and 10-171).

Figure 4-62 Diffuse anterior scleritis demonstrating characteristic deep red, violaceous hue.

Figure 4-63 Nodular anterior scleritis. Note the elevated nodule within the deep, red, sectoral injection.

Figure 4-64 Necrotizing scleritis demonstrating thinning of sclera superiorly with increased visibility of the underlying blue uvea.

Figure 4-65 Scleromalacia perforans demonstrating characteristic blue appearance due to visible uvea underneath thin sclera.

Differential Diagnosis

Episcleritis, iritis, phlyctenule, retrobulbar mass, myositis, scleral ectasia, and staphyloma.

Evaluation

• Complete ophthalmic history and eye exam with attention to pattern of conjunctival and scleral injection and failure of area to blanch with topical vasoconstrictor (phenylephrine 2.5%), cornea, anterior chamber, and ophthalmoscopy.

• Lab tests: Complete blood count (CBC), rheumatoid factor (RF), antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), Venereal Disease Research Laboratory (VDRL) test, fluorescent treponemal antibody absorption (FTA-ABS) test, PPD and controls, and chest radiographs.

• B-scan ultrasonography: Thickened sclera and “T-sign” in posterior scleritis (see Figure 10-172).

• Medical consultation.

Management

• Depending on severity, consider using one or a combination of the following:

• Systemic NSAID (indometacin 50 mg po bid to tid, Dolobid 500 mg po qd to bid, Celebrex 200 mg po bid).

• Oral steroids (prednisone 60–100 mg po qd); check PPD and controls, blood glucose, and chest radiographs before starting systemic steroids. Note: topical steroids and NSAIDs are ineffective.

• Add H₂-blocker (ranitidine [Zantac] 150 mg po bid) or proton pump inhibitor when administering systemic NSAIDs or steroids.

• Immunosuppressive agents (see Anterior Uveitis section in Chapter 6) in severe cases; should be administered by a uveitis specialist.

• Sub-Tenon’s steroid injection is contraindicated.

• May require surgery (patch graft) for globe perforation.

Prognosis

Depends on etiology; poor for necrotizing form, which may perforate; scleromalacia perforans rarely perforates; recurrences are common.

Scleral Discoloration

Definition

Alkaptonuria (Ochronosis)

Recessive inborn error of metabolism (accumulation of homogentisic acid) causing brown pigment deposits in eyes, ears, nose, joints, and heart; triangular patches in interpalpebral space near limbus, pigmentation of tarsus and lids.

Ectasia (Staphyloma)

Congenital, focal area of scleral thinning usually near limbus; underlying uvea is visible and may bulge through defect; perforation is uncommon.

Figure 4-66 Scleral and corneal staphylomas with bulging blue appearance.

Osteogenesis Imperfecta (Autosomal Dominant)

Congenital disorder of type I collagen; mapped to chromosome 17q21.3-22.1 (COL1A1 gene) and chromosome 7 (COL1A2 gene). Sclera is thin and appears blue due to underlying uvea (also found in Ehlers–Danlos syndrome). If patient develops scleral icterus, then the sclera may appear green.