Fuchs' heterochromic cyclitis and retinal vascular abnormalities in progressive hemifacial atrophy

We report a case of progressive hemifacial atrophy with a combination of ipsilateral Fuchs' heterochromic cyclitis and retinal vascular abnormalities. Recently, evidence was found for a neurovascular defect in hemifacial atrophy. Our case not only supports the (clinical) association between Fuchs' heterochromic cyclitis and hemifacial atrophy, but the retinal vascular abnormalities found in this patient add further support to the existence of a neurovascular defect. These findings and our short review of the literature point to the hypothesis of a common sympathetic defect, implicated in the aetiology of both Fuchs' heterochromic cyclitis and progressive hemifacial atrophy. It has to be borne in mind, however, that Fuchs' heterochromic cyclitis has been reported in association with other diseases. It seems likely that although Fuchs' heterochromic cyclitis is a single clinical entity, it may have more than one cause.