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The treatment of refractory angle-closure glaucoma in a patient with X-linked juvenile retinoschisis

Low, S; Mohamed, R; Ting, M; Webster, AR; Garway-Heath, DF; (2018) The treatment of refractory angle-closure glaucoma in a patient with X-linked juvenile retinoschisis. Ophthalmic Genetics , 39 (5) pp. 625-627. 10.1080/13816810.2018.1490961. Green open access

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Abstract

X-Linked Retinoschisis (XLRS) is a common genetically determined form of macular degeneration affecting young males. XLRS is due to mutations in the RS1 gene located on chromosome Xp22 which codes for retinoschisin and is estimated to affect between 1:5000 to 1:20000 individuals worldwide. We report a case of refractory angle-closure glaucoma in a thirty-nine-year-old Caucasian man with atypical XLRS. The patient presented with a two-day history of left eye pain, acutely reduced vision and a nine-month history of hemicranial pain. Examination identified left intraocular pressure (IOP) of 52mmHg. Gonioscopy confirmed complete angle closure. Following failure of medical management and persistently raised left IOP (43-46mmHg), the patient underwent left phacoemulsification and intraocular lens insertion without complication. After surgery, his IOP reduced to 10-14mmHg on all follow up examinations without the need for glaucoma drops. His iridocorneal angle remained open and vision improved to 20/100. Our case demonstrates the additional role of lens surgery in the treatment of secondary angle-closure glaucoma in the presence of an inherited retinal dystrophy. All patients with inherited retinopathy presenting with a headache or eye pain should undergo gonioscopic examination to exclude angle-closure glaucoma.

Type: Article
Title: The treatment of refractory angle-closure glaucoma in a patient with X-linked juvenile retinoschisis
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1080/13816810.2018.1490961
Publisher version: https://doi.org/10.1080/13816810.2018.1490961
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Angle-closure glaucoma, X-linked retinoschisis, cataract surgery, lens extraction
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/10059543
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