Progressive hemifacial atrophy (Parry-Romberg disease)

Abstract

Hemifacial atrophy (Parry-Romberg syndrome) is characterized by slowly progressive atrophy of one side of the face, primarily involving the subcutaneous tissue and fat. The onset is usually in the first two decades. Ophthalmic involvement is common; the most frequent abnormality is progressive endophthalmos with subsequent changes in the palpebral fissure. Pupillary disturbances, heterochromia, uveitis, and restrictive strabismus have also been frequently reported. We describe six cases that manifest a wide spectrum of ocular and systemic findings. They are noteworthy in that all exhibit pigmentary disturbances of the ocular fundus, a finding rarely reported. Another unusual ocular manifestation in one patient was an acquired partial third nerve palsy on the unaffected side.