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Abstract #65983 Published in IGR 17-3
WHITE ANNULAR RETINAL DYSTROPHY WITH SEVERE GLAUCOMA: A New Autosomal Dominant Condition
Morizot R; van Schooneveld MJ; Morizot ERetina (Philadelphia, Pa.) 2016; 36: 619-623
PURPOSE: To report a family with a previously unreported characteristic retinal dystrophy and glaucoma. METHODS: Seven family members were diagnosed with an atypical retinal dystrophy and open-angle glaucoma with rapid evolution. Ophthalmic examination, fluorescein angiography, color photography, optic coherence tomography, central visual-field examination, and ultrasonography were performed. RESULTS: Of the 7 patients, 3 had 360° of peripheral white retina and a broad white ring around the optic disc. In three others, it was not possible to observe the peripheral retina, but they also showed a white retinal ring around the optic disc. One patient showed posterior synechiae and iris neovascularization in one eye. The 37-year-old uncle of the proband had a probably related maculopathy. Five patients had severe glaucoma, and the youngest showed borderline intraocular pressure. CONCLUSION: The authors report a new dominant retinal dystrophy associated with open-angle glaucoma. The early onset and rapidly progressive glaucoma of the patients is atypical.
*Department of Ophthalmology, Instituto Benjamin Constant, Rio de Janeiro, Brazil; and †Academic Medical Centre, Department of Ophthalmology, Amsterdam, the Netherlands.
Full articleClassification:
9.4.5.5 Other (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.5 Glaucomas associated with disorders of the retina, choroid and vitreous)