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General Information

Salzmann nodular degeneration is a fairly common, unilateral or bilateral condition characterized by smooth creamy-white elevated lesions of the cornea. Found much more commonly in women.

Symptoms

Often asymptomatic. May affect vision if it involves the paracentral or central cornea. It may cause a foreign-body sensation if the lesions become very elevated.

Signs

Critical

Single or multiple, discrete, white or gray-white or occasionally bluish, smooth, elevated nodules on the surface of the cornea, typically peripheral but can be central (see Figure 4.10.1).

Figure 4.10.1: Salzmann nodular degeneration with an iron line.

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Other

Long-standing nodules may have iron pigment deposition in the epithelium at the base of the nodule. Corneal topography can reveal significant central irregular astigmatism from fairly mild and peripheral nodules.

Differential Diagnosis

Etiology

A subepithelial deposition of disorganized extracellular matrix from unknown cause. It is often found in eyes with a history of chronic keratopathy, such as interstitial keratitis (IK), vernal keratoconjunctivitis, dry eye syndrome, phlyctenulosis, and trachoma, and in eyes with a history of chronic contact lens wear, trauma or corneal surgery, but frequently appears in otherwise normal eyes.

Workup

  1. History: Chronic eye disease? Previous ocular trauma or surgery? Contact lens wear?

  2. Complete ophthalmic examination, including IOP measurement, and fluorescein staining for epithelial defects.

  3. Consider corneal topography looking for corneal irregularity.

Treatment

  1. Mild cases are observed or treated with lubrication.

  2. If the nodules are causing symptoms, they may be treated with superficial keratectomy with a blade or excimer laser PTK. Topical mitomycin C at the time of surgical excision may decrease the rate of recurrence. Rarely, if severe, it may require a lamellar keratoplasty.

Follow-Up

Asymptomatic patients can be checked every 1 to 2 years. Symptomatic patients should be seen more frequently. Nodules should be measured at follow-up visits to look for progression. Consider checking corneal topography routinely to monitor the degree of irregular astigmatism.