Posters

A rare association of foveoschisis with gyrate atrophy

Poster Details

First Author: M.Teke TURKEY

Co Author(s):    K. Tekin   M. Citirik                          

Abstract Details



Purpose:

Gyrate is a rare, genetically determined, autosomal recessive metabolic disorder characterized by multiple sharply demarcated circular or oval areas of chorioretinal atrophy in the mid-periphery of the fundus. A kind of macular involvement have been reported in this disease including cystoid macular edema, epimacular membrane, macular hole, and choroidal neovascularization. However, the association of foveoschisis with gyrate atrophy is very rare. This poster aimed to report a rare association of foveoschisis with gyrate atrophy (GA).

Setting:

Ulucanlar Eye Training and Research Hospital.

Methods:

A case report. 17 year-old female with an unusual appearance on fundus photography and spectralis optical coherence tomography (OCT) was presented.

Results:

The patient's visual acuities were 20/100 with -2.00 spherical diopters (D) in the right eye and 20/80 with -2.50 spherical D in the left eye. While the anterior segment examinations were unremarkable for both eyes, the fundus examination of the patient demonstrated multiple bilateral, sharply defined, and scalloped chorioretinal atrophy areas in the mid-peripheral zones. Fundus fluorescein angiography did not show any leakage even in late phases for both eyes. The wide-field OCT scans of both eyes disclosed that increased central macular thicknesses with widespread hyporeflective spaces separated by multiple linear bridging elements in inner nuclear and inner plexiform layers in addition to retinal defects at outer nuclear and outer plexiform layers. Moreover, elevated level of plasma ornithine (967 Mmol/L) was detected, establishing the diagnosis of GA. Additionally, ocular examinations revealed no specific findings such as optic pits, myopic degeneration with staphyloma, or vitreoretinal traction.

Conclusions:

Foveoschisis may rarely accompany GA; OCT is a helpful diagnostic imaging technique to identify this concomitant condition.

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