BASICS

DESCRIPTION

Salzmann’s nodules are creamy white, occasionally with a yellow or blue-tinged, smooth, elevated lesion(s) on the surface of the cornea.

EPIDEMIOLOGY

Incidence

• Uncommon but exact incidence is unknown

• Tends to occur in middle age

Prevalence

Much more prevalent in women than in men

RISK FACTORS

• Ocular surface inflammation, such as from meibomian gland dysfunction (MGD), dry eye syndrome (DES), phlyctenular keratitis, vernal keratoconjunctivitis, trachoma, interstitial keratitis, and trauma, such as long-term contact lens wear

• Most commonly idiopathic

GENERAL PREVENTION

Treat underlying conditions

ETIOLOGY

Thought to be related to low-grade chronic inflammation

COMMONLY ASSOCIATED CONDITIONS

• See risk factors above.

• Salzmann’s has also been associated with epithelial basement membrane dystrophy and after corneal surgery.

DIAGNOSIS

HISTORY

• Generally asymptomatic

• May have symptoms of foreign body sensation, pain, redness, and tearing

• As nodules reach toward the visual axis, the vision may be mildly to moderately affected.

PHYSICAL EXAM

• Slit lamp examination reveals single or multiple, elevated creamy, yellow- or blue-white corneal nodules.

• Although typically located in the corneal periphery or midperiphery, the nodules may also be paracentral or central.

DIAGNOSTIC TESTS & INTERPRETATION

Imaging

Initial approach

Not required if vision is unaffected

Follow-up & special considerations

Consider corneal topography to evaluate for irregular astigmatism if there are visual complaints.

Pathological Findings

• Collagen plaques with hyaline between epithelium and Bowman’s layer

• Bowman’s layer may be missing or damaged underlying the lesion.

DIFFERENTIAL DIAGNOSIS

• Climatic droplet keratopathy

• Corneal amyloidosis

• Corneal keloid

TREATMENT

MEDICATION

First Line

• If asymptomatic, no treatment is required.

• To decrease the risk of progression, underlying conditions can be treated.

• MGD and DES should be treated if present.

• Proper contact lens fit, wearing schedule, and care should be evaluated.

Second Line

Foreign body sensation or pain is treated with increased lubrication with artificial tears, gels and ointments, cyclosporine 0.05% drops, and punctal plugs.

ADDITIONAL TREATMENT

General Measures

Salzmann’s nodules may remain stable for years or may slowly progress.

Issues for Referral

When discomfort or visual symptoms are not responding to conservative medical therapy, referral to a corneal specialist may be warranted.

Additional Therapies

A rigid gas permeable or hybrid contact lens may occasionally be useful in improving vision in patients with irregular astigmatism, who are not good candidates for surgical excision of the nodule(s).

SURGERY/OTHER PROCEDURES

• Surgical excision of the nodules is recommended when comfort or vision symptoms are not relieved by medical therapy.

• Excision can often be accomplished by simple removal with a blade, being careful not to incise into Bowman’s layer.

• Excimer laser phototherapeutic keratectomy (PTK) is often used to remove these lesions.

• Recurrences of the nodules after excision may be reduced with the use of intraoperative mitomycin C.

• Rarely, a lamellar or penetrating keratoplasty is required for severe disease or multiple recurrences.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Asymptomatic patients are generally seen every year.

• Symptomatic patients are seen more frequently based on their symptoms.

• Patients are seen every few days after surgical excision to make sure that the cornea is healing well.

PATIENT EDUCATION

Patients should return to their eye doctor should they develop pain or decreased vision.

PROGNOSIS

• Very good

• Lesions are often stable for many years, also they may slowly enlarge.

• Lesions may recur after surgical excision; repeat excision is often successful.

COMPLICATIONS

Patients may develop epithelial defects or recurrent erosions related to the nodules, which could become infected.

ADDITIONAL READING

• Farjo AA, Halperin GI, Syed N, et al. Salzmann’s nodular corneal degeneration clinical characteristics and surgical outcomes. Cornea 2006;25:11–15.

• Das S, Link B, Seitz B. Salzmann’s nodular degeneration of the cornea A review and case series. Cornea 2005;24:772–777.

• Bowers PJ, Price MO, Zeldes SS, et al. Superficial keratectomy with mitomycin-C for the treatment of Salzmann’s nodules. J Cataract Refract Surg 2003;29:1302–1306.

• Marcon AS, Rapuano CJ. Excimer laser phototherapeutic keratectomy retreatment of anterior basement dystrophy and Salzmann’s nodular degeneration with topical mitomycin C. Cornea 2002;21:828–830.

CODES

ICD9

371.46 Nodular degeneration of cornea

CLINICAL PEARLS

• Salzmann’s nodular degeneration is not rare, especially among middle-aged women.

• Salzmann’s nodular degeneration should be considered in anyone with creamy white, yellow, or bluish, smooth, elevated corneal nodule(s).

• Medical treatment is often successful for lesions causing foreign body sensation, but surgical excision is generally required for patients with frank pain (rare) or decreased vision (common).

• Although surgical excision is quite successful, nodules may recur.

• Any nodules causing significant irregular astigmatism should be removed prior to cataract surgery to obtain the most accurate keratometry readings.

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