A Very Rare Association of Fuchs Heterochromic Uveitis and Ectropion Uvea in Usher Syndrome

Leila Rezaei; Rashed Ahmadyani

doi:10.4103/abr.abr_286_20

A Very Rare Association of Fuchs Heterochromic Uveitis and Ectropion Uvea in Usher Syndrome

Adv Biomed Res. 2021 Dec 25:10:50. doi: 10.4103/abr.abr_286_20. eCollection 2021.

Authors

Leila Rezaei¹, Rashed Ahmadyani²

Affiliations

¹ Associate Professor of Ophthalmology, Clinical Research Development Center, Imam Khomeini and Mohammad Kermanshahi and Farabi Hospitals, Kermanshah University of Medical Sciences, Kermanshah, Iran.
² Students Research Committee, Kermanshah University of Medical Sciences, Kermanshah, Iran.

Abstract

Usher syndrome is a heterogeneous genetic disease that is the most common cause of hereditary blindness-deafness. This syndrome is the most prevalent syndrome associated with retinitis pigmentosa. A 25-year-old man referred to us with hearing loss and vision diminution since childhood which has gradually worsened. Visual acuity of both eyes was 20/100. Slit lamp examination of the left eye revealed endothelial stellate keratic precipitates, mild anterior chamber reaction, iris heterochromia, ectropion of uvea, and mild posterior subcapsular cataract. There were also no crypts and abnormal vessels in the left eye iris. His intraocular pressure was 14 mmHg in the right eye and 18 mmHg in the left one. Funduscopy demonstrated waxy pallor optic nerve, marked arterial narrowing, and retinal bone spicule pigment formation in both eyes. We report for the first time a very rare association between Usher syndrome, Fuchs heterochromic uveitis (FHU), and ectropion uvea. To our knowledge, no association has been reported between ectropion uvea, FHU, and Usher syndrome.

Keywords: Cataract; deafness; heterochromia iridis; iridocyclitis; retinitis pigmentosa; usher syndrome; uveitis.

Publication types

Case Reports