WHITE ANNULAR RETINAL DYSTROPHY WITH SEVERE GLAUCOMA A New Autosomal Dominant Condition

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 degrees 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