Abstract
Abstract: :
Purpose: To report the incidence of Stargardt macular dystrophy in an adult suffering from recurrent iridocyclitis, and provide possible correlations of those two eye conditions. Methods: A forty–year–old man was identified with metamorphopsia and bilateral visual loss over a period of months. He underwent complete ophthalmological work–up including fluorescein angiogram, optical coherence tomography (OCT), visual field testing and detailed electrophysiology. Results: The patient had a history of recurrent iridocyclitis associated with ankylosing spondylitis. Fundus appearance was normal during the twelve years of regular follow–up. He was under systemic medication for his spondylitis, which was well controlled. Iridocyclitis responded well to local corticosteroid treatment. His family history was nonsignificant for any eye problems. Gradual bilateral visual loss over eight months with subsequent stabilization at a visual level of 20/40 was noted. Fundus examination revealed a localized atrophic–appearing foveal lesion with a garland of yellow–white flecks at the level of retinal pigment epithelium at the posterior pole. On fluorescein angiography hyperfluorescence was noticed at the macular area surrounded by a "dark choroid". OCT of the macula revealed central atrophy. Visual fields showed a central scotoma. Rod and cone ERG a– and b–wave amplitudes were normal. Pattern ERG demonstrated marked central retinal dysfunction. Conclusions: The incidence of Stargardt macular dystrophy in that patient who suffered from a form of uveitis, other than a simple coincidence may well be a source of hypotheses about their correlation. Possible aspects of their etiopathogenic association are discussed. There are maybe more genetically mild similar cases that never manifest, due to a lack of stimuli necessary to trigger specific cellular pathways.
Keywords: retinal degenerations: hereditary • retinal pigment epithelium