title: Ultrawide field fluorescein angiogram in a family with gyrate atrophy and foveoschisis.
abstract: Gyrate atrophy of choroid and retina is an autosomal recessive condition characterized by peripheral multiple sharp areas of chorioretinal atrophy which become confluent with age. Macula and central vision is typically involved late in the disease. Macular involvements such as cystoid macular edema, epimacular membrane, and choroidal neovascularization have been reported in gyrate atrophy. In this report, we present a family with diminished central vision presenting within 8 years of age. All of three siblings had typical peripheral chorioretinal atrophic lesions of gyrate atrophy and hyperornithinemia. On spectral domain optical coherence tomography, two of elder siblings showed macular edema. Hyporeflective spaces appeared to extend from outer nuclear layer to the inner nuclear layer level separated by multiple linear bridging elements in both eyes. Ultrawide field fluorescein angiogram (UWFI) even in late phase did not show any leak at macula suggesting foveoschisis. Foveoschisis in gyrate atrophy has not been reported before.
authors.affiliation: Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Koushik
authors.initials: K
authors.lastname: Tripathy
authors.affiliation: Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Rohan
authors.initials: R
authors.lastname: Chawla
authors.affiliation: Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Yog Raj
authors.initials: YR
authors.lastname: Sharma
authors.affiliation: Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Varun
authors.initials: V
authors.lastname: Gogia
keywords: Cystoid macular edema; fundus dystrophies; macular dystrophy; maculoschisis; scanning laser ophthalmoscope