RGP Scleral Lenses for Stevens-Johnson Syndrome

BY ROBERT CAMPBELL, M.D. & PATRICK CAROLINE, C.O.T., F.A.A.O.
MAY 1997

Erythmia multiforme, or Stevens-Johnson syndrome, is a severe dermatologic condition traditionally associated with a hypersensitivity to systemic drugs such as sulfonamides, barbiturates, penicillin, phenylbutazone or mercurials. However, it has been known to occur following respiratory infection, herpes simplex infection, measles infection and smallpox vaccination, and occurs most commonly in children and young or middle-aged adults. The systemic involvement is usually sudden and stormy with fever, malaise and respiratory symptoms followed by the eruption of multiple skin lesions.

Once the systemic disease has resolved, the ocular findings begin. These can include severe corneal and conjunctival scarring, keratoconjunctivitis sicca, corneal vascularization, keratinization of the cornea and lid margins, symblepharon, trichiasis and entropion.

TREATMENT

Traditional treatment for the disease is difficult and often unsuccessful. Systemic or topical steroids can be used, but secondary infection and corneal perforations are a constant threat. Medical treatment must be supplemented with lid hygiene and frequent (every hour) application of preservative-free lubricants. Maintenance of the fornices requires frequent lysis of the symblepharon in the acute phase. In the advanced stages, a PMMA symblepharon ring or a scleral shell will help inhibit symblepharon formation, allowing the fornices to be maintained.

CASE HISTORY

A 61-year-old woman who developed Stevens-Johnson syndrome in 1991 while being treated with gold therapy for pemphigus subsequently developed a series of corneal ulcers in her left eye, causing corneal thinning and perforation. Numerous surgical interventions and progression of the disease resulted in severe sicca, extensive conjunctival and corneal scarring, keratinization of the ocular surfaces and lid margins, corneal vascularization, entropion, trichiasis and symblepharon with a complete loss of the fornices (Fig. 1). Visual acuity OS was hand motion.

FIG. 1: OCULAR MANIFESTATIONS OF STEVENS-JOHNSON SYNDROME.

FIG. 2: A HIGH DK SCLERAL LENS OD FOR STEVENS-JOHNSON SYNDROME.

The patient's right eye had fared slightly better with traditional treatment, which included extensive lid hygiene, frequent preservative-free lubrication, oral and topical steroids and antibiotics. However, the right eye had developed significant vascularization and corneal thinning with recurrent epithelial defects. Best corrected visual acuity was 20/400. Due to the patient's deteriorating condition, we referred her to the Boston Foundation for Sight where she was fitted with a high Dk (110) scleral lens (Fig. 2). The lens, developed by Perry Rosenthal, M.D., was approved by the FDA in 1994 for visual rehabilitation of damaged corneas from injury, surgery or disease.

Immediately following the fitting, visual acuity improved to 20/50. The patient has been able to wear the lens during all waking hours with significant improvement in her ocular comfort and external findings. The lens has provided excellent protection of the ocular surfaces from the scarred lids and aberrant lashes with no episodes of recurrent epithelial defects. The lens is removed approximately every four hours for cleaning and exchange of preservative-free Unisol saline.

High Dk scleral lenses could be beneficial for a variety of conditions including ocular pemphigoid, advanced keratoconus, pellucid marginal degeneration, interstitial keratitis, trachoma, keratoglobus, Terrien's marginal degeneration, rosacea keratitis, chemical burns and traumatic scarring. CLS

Dr. Campbell is medical director of the Park Nicollet Contact Lens Clinic & Research Center, Minnetonka, Minn. Patrick Caroline is an assistant professor of optometry at Pacific University, Forest Grove, Ore., and director of contact lens research at Oregon Health Sciences University in Portland.