Coats-like lesions in Usher syndrome type II

Graefes Arch Clin Exp Ophthalmol. 2004 Mar;242(3):265-7. doi: 10.1007/s00417-003-0818-2. Epub 2003 Dec 3.

Abstract

Background: An unusual case of Usher syndrome type II associated with bilateral Coats-like exudative retinopathy is described.

Methods: A 14-year-old boy with congenital sensorineural deafness and normal vestibular functions presented with a recent history of night blindness. He was followed for 3 years with fundus photography, intravenous fluorescein angiography, electroretinography and audiometric testings. His parents refused any form of treatment.

Results: Fundoscopy showed bilateral retinitis pigmentosa and a single focus of subretinal exudation and overlying telangiectatic retinal vessels inferotemporal to the vascular arcade in the right eye. He had bilateral mild macular edema. A year later, a similar lesion developed inferotemporally in the left fundus. Electroretinography responses, particularly the rod-mediated signals, were significantly reduced. Audiometric studies documented hearing loss in high frequencies. His visual acuity declined from 20/40 to 20/80 RE and from 20/80 to 20/100 LE during follow-up. No new lesions developed.

Conclusions: Coats-type exudative lesions may develop in patients with Usher syndrome type II. Although left untreated, only a minimal increase in exudation occurred over 3 years.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Audiometry
  • Electroretinography
  • Exudates and Transudates
  • Fluorescein Angiography
  • Hearing Loss, Sensorineural / classification
  • Hearing Loss, Sensorineural / complications*
  • Hearing Loss, Sensorineural / congenital
  • Humans
  • Macular Edema / diagnosis
  • Macular Edema / etiology*
  • Male
  • Night Blindness / classification
  • Night Blindness / complications*
  • Night Blindness / congenital
  • Retinal Diseases / diagnosis
  • Retinal Diseases / etiology*
  • Retinal Vessels / pathology*
  • Retinitis Pigmentosa / complications*
  • Telangiectasis / diagnosis
  • Telangiectasis / etiology*