P 604
Corneal decompensation in a patient with Parry-Romberg Syndrome
M. Wollenhaupt, C. D. Quentin, M. Vogel
Background: The Parry-Romberg Syndrome is a rare disease. It was first described by C. H. Parry in 1825. The German Neurologist M. H. von Romberg described the full syndrome in 1846. The term progressive facial hemiatrophy is used as a synonym. Diagnosis, therapy and outcome will be discussed in a case report.
Case Report: A female patient was admitted to our hospital with the diagnosis of left corneal decompensation. Visual acuity was 0,05 OD and 0,08 OS. The amblyopia of the OD was caused by strabismus. Since 1991 the patient had been suffering from recurrent iridocyclitis with heterochromic iris and secondary glaucoma. Striking was an asymmetric face ("en coup de sabre"-form) and an enophthalmus of the left side. The patient´s history revealed epilepsy and hypertonus. With support of imaging techniques and neurological and dermatological consultation the diagnosis of Parry-Romberg syndrome was established. Because the corneal state deteriorated we had to perform a penetrating keratoplasty and a glaucoma operation.
Discussion: The Parry-Romberg syndrome consists of five diagnostic criteria. Our patient presented the classical features of the disease. Ocular findings are various, including enophthalmus, iricocyclitis and secondary glaucoma. To our knowledge a corneal decompensation within this disease has not yet been described in the literature. So far therapy is only symptomatically possible. The pathogenesis of the disease is still unknown. Frequently discussed is the hypothesis of a trophic nervous malfunction due to damage to the sympathetic nervous system.
Eye Clinic of the Georg-August-University in Goettingen, Robert-Koch-Str.40, D-37075 Goettingen