Coats-like lesions in Usher syndrome type II.
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.
Kiratli
Hayyam
H
Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey. hkiratli@hacettepe.edu.tr
Oztürkmen
Cem
C
D000293:Adolescent; D001299:Audiometry; D004596:Electroretinography; D005122:Exudates and Transudates; D005451:Fluorescein Angiography; D006319:Hearing Loss, Sensorineural / Q000145:classification / Q000150:complications* / Q000151:congenital; D006801:Humans; D008269:Macular Edema / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D009755:Night Blindness / Q000145:classification / Q000150:complications* / Q000151:congenital; D012164:Retinal Diseases / Q000175:diagnosis / Q000209:etiology*; D012171:Retinal Vessels / Q000473:pathology*; D012174:Retinitis Pigmentosa / Q000150:complications*; D013684:Telangiectasis / Q000175:diagnosis / Q000209:etiology*