title: WHITE ANNULAR RETINAL DYSTROPHY WITH SEVERE GLAUCOMA: A New Autosomal Dominant Condition.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: *Department of Ophthalmology, Instituto Benjamin Constant, Rio de Janeiro, Brazil; and †Academic Medical Centre, Department of Ophthalmology, Amsterdam, the Netherlands.
authors.forename: Rodrigo
authors.initials: R
authors.lastname: Morizot
authors.forename: Mary J
authors.initials: MJ
authors.lastname: van Schooneveld
authors.forename: Eduardo
authors.initials: E
authors.lastname: Morizot
mesh_terms: D000293:Adolescent; D000328:Adult; D015785:Eye Diseases, Hereditary / Q000175:diagnosis* / Q000235:genetics; D005260:Female; D005451:Fluorescein Angiography; D005799:Genes, Dominant; D005902:Glaucoma, Open-Angle / Q000175:diagnosis* / Q000235:genetics; D006068:Gonioscopy; D006801:Humans; D007429:Intraocular Pressure; D008297:Male; D010375:Pedigree; D058499:Retinal Dystrophies / Q000175:diagnosis* / Q000235:genetics; D041623:Tomography, Optical Coherence; D014065:Tonometry, Ocular; D058609:Visual Field Tests; D014794:Visual Fields; D055815:Young Adult