Severe Hypotony Associated With Parry-Romberg Syndrome.

Abstract

Parry-Romberg syndrome is a rare inflammatory disorder characterized by progressive hemifacial atrophy and ocular involvement. Two patients with Parry-Romberg syndrome who had mild heterochromic uveitis but developed profound ocular hypotony were evaluated. A 17-year-old girl and a 32-year-old woman with Parry-Romberg syndrome developed chronic uveitis with gradual worsening of intraocular pressure to 0 mm Hg. For the first time, ultrasound biomicrosopy found evidence of inflammation of the ipsilateral ciliary muscle in patients with hemifacial atrophy. The profound hypotony concomitant with ciliary body edema in two patients with Parry-Romberg syndrome provides a clue linking the systemic disease to the ocular findings.