title: Ultrawide-field fundus photography of the first reported case of gyrate atrophy from 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.
authors.affiliation: Department of Ophthalmology, Cairns Hospital, Cairns, QLD, Australia.
authors.forename: Thomas P
authors.initials: TP
authors.lastname: Moloney
authors.affiliation: Department of Ophthalmology, Cairns Hospital, Cairns, QLD, Australia.
authors.forename: Stephen
authors.initials: S
authors.lastname: O'Hagan
authors.affiliation: City Eye Centre, Brisbane, QLD, Australia ; Associate Professor of Ophthalmology, School of Medicine, University of Queensland, Brisbane, QLD, Australia.
authors.forename: Lawrence
authors.initials: L
authors.lastname: Lee
keywords: angiography; gyrate atrophy; hyperornithinemia; retinal photography; ultrawide-field retinal photography