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Ultrawide-field fundus photography of the first reported case of gyrate atrophy from Australia

Authors Moloney T, O'Hagan S, Lee L

Received 17 March 2014

Accepted for publication 19 April 2014

Published 20 August 2014 Volume 2014:8 Pages 1561—1563

DOI https://doi.org/10.2147/OPTH.S64248

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 3


Thomas P Moloney,1 Stephen O’Hagan,1 Lawrence Lee2,3

1Department of Ophthalmology, Cairns Hospital, Cairns, QLD, Australia; 2City Eye Centre, Brisbane, QLD, Australia; 3Associate Professor of Ophthalmology, School of Medicine, University of Queensland, Brisbane, QLD, Australia

Abstract: Gyrate atrophy of the choroid and retina is a rare chorioretinal dystrophy inherited in an autosomal recessive pattern. We describe the first documented case of gyrate atrophy from Australia in a 56-year-old woman with a history of previous diagnosis of retinitis pigmentosa and worsening night vision in her right eye over several years. She was myopic and bilaterally pseudophakic, and fundus examination revealed pale optic discs and extensive peripheral chorioretinal atrophy exposing bare sclera bilaterally with only small islands of normal-appearing retina at each posterior pole. Visual field testing showed grossly constricted fields, blood testing showed hyperornithinemia, and further questioning revealed consanguinity between the patient’s parents. We then used the patient’s typical retinal findings of gyrate atrophy to demonstrate the potential use of ultrawide-field fundus photography and angiography in diagnosis and monitoring response in future treatment.

Keywords: gyrate atrophy, ultrawide-field retinal photography, angiography, retinal photography, hyperornithinemia

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