title: FUNCTIONAL AND ANATOMICAL OUTCOMES OF CHOROIDAL NEOVASCULARIZATION COMPLICATING BEST1-RELATED RETINOPATHY.
abstract: OBJECTIVE
  To describe the presenting features and functional outcomes in a series of patients with choroidal neovascular membrane complicating BEST1-related retinopathy (Best disease and autosomal recessive bestrophinopathy).
  
  
  METHODS
  Retrospective review of consecutive cases at a tertiary care eye hospital. Patients were identified retrospectively over an 11-year period. Records were reviewed to extract demographic as well as functional and anatomical outcome data.
  
  
  RESULTS
  Fourteen eyes of 12 patients were identified (11 Best disease and 1 autosomal recessive bestrophinopathy). Median follow-up was 2.8 years (range 0.8-6). The median age at choroidal neovascular membrane discovery was 15.5 years (range 6-72). Choroidal neovascular membranes were active early in the disease course before vitelliruption. Seven eyes were treated with intravitreal bevacizumab, 7 eyes were monitored by observation alone. On average, patients required a single treatment (median = 1, range 1-10). The median gain in visual acuity was greater in the treated versus the observed group-0.46 versus 0.17 decimalized units of Snellen acuity, respectively (P < 0.05 Mann-Whitney U test). Although a significant reduction in central macular thickness was evident in both groups, 150 μm (treated) and 104 μm (observed), active treatment was not associated with greater thinning than observation (P > 0.05 Mann-Whitney U test).
  
  
  CONCLUSIONS
  There is a high rate of spontaneous recovery of BEST1-related choroidal neovascular membrane, and overall the authors observed a gain in visual acuity associated with a reduction in central macular thickness. Active treatment, here with intravitreal bevacizumab, is associated with better functional outcomes than observation alone.
authors.affiliation: *University College London Institute of Ophthalmology, University College London, London, United Kingdom; †Medical Retina Service, Moorfields Eye Hospital, London, United Kingdom; ‡Department of Ophthalmology, Leeds Institute of Molecular Medicine, St James' University Hospital, Leeds, United Kingdom; and §Department of Ophthalmology, University of California San Francisco Medical School, San Francisco, California.
authors.forename: Kamron N
authors.initials: KN
authors.lastname: Khan
authors.forename: Omar A
authors.initials: OA
authors.lastname: Mahroo
authors.forename: Farrah
authors.initials: F
authors.lastname: Islam
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Webster
authors.forename: Anthony T
authors.initials: AT
authors.lastname: Moore
authors.forename: Michel
authors.initials: M
authors.lastname: Michaelides
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002829:Choroid / Q000473:pathology*; D020256:Choroidal Neovascularization / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012160:Retina / Q000473:pathology*; D012189:Retrospective Studies; D013997:Time Factors; D041623:Tomography, Optical Coherence; D014792:Visual Acuity; D057826:Vitelliform Macular Dystrophy / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D055815:Young Adult