Abstract • A 22-year-old patient with newly diagnosed facioscapulohumeral (FSH) muscular dystrophy had a macular lesion in her right eye and poor central vision, which had been present since early childhood. Fluorescein angiographic examination revealed bilateral peripheral vessel closure, peripheral retinal telangiectasis, and hyperfluorescence in both foveae. This widespread vascular abnormality was deemed responsible for her macular disease. Her mother, brother, and sister, all of whom are affected by varying degrees of FSH muscular dystrophy and clinical deafness, also have abnormal retinal vasculature, as determined by fluorescein angiography. However, none had related visual symptoms and two showed no ophthalmoscopic evidence of vascular abnormalities. In young patients with unexplained retinal vascular lesions, the diagnosis of FSH muscular dystrophy should be considered. Similarly, young patients with FSH muscular dystrophy should be examined for sight-threatening and potentially treatable vascular retinopathy. References 1. Tyler FH, Stephens FE: Studies in disorders of muscle: II. Clinical manifestations and inheritance of facioscapulohumeral dystrophy in a large family . Ann Intern Med 1950;32:640-660.Crossref 2. Carroll JE, Brooke MH: Infantile facioscapulohumeral dystrophy , in Serratrice G, Roux H (eds): Peroneal Atrophies and Related Disorders . New York, Masson Publishing USA Inc, 1979, pp 305-318. 3. Taylor DA, Carroll JE, Smith ME, et al: Facioscapulohumeral dystrophy associated with hearing loss and Coats syndrome . Ann Neurol 1982;12:395-398.Crossref 4. Wulff JD, Lin JT, Kepes JJ: Inflammatory facioscapulohumeral muscular dystrophy and Coats syndrome . Ann Neurol 1982;12:398-401.Crossref 5. Small RG: Coats' disease and muscular dystrophy . Ophthalmology 1968;72:225-231. 6. Coats G: Forms of retinal disease with massive exudation . 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Archives of Ophthalmology – American Medical Association
Published: Nov 1, 1985
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