Nigerian Journal of Ophthalmology

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X-linked juvenile retinoschisis: A case report

Nouhoum Guirou, Fatoumata Sylla, Yakoura H. Abba Kaka, Japhet Thera, Seydou Bakayoko, Amassagou Dougnon, Sanoussi Bamani, Jeannette Traoré


X-linked juvenile retinoschisis is a hereditary macular dystrophy that is transmitted in the X-linked recessive mode. Clinical signs include a macular star with or without peripheral retinoschisis responsible for decreased visual acuity. This study dealt with a 12-year-old boy who came in for a consultation for progressive decline in visual acuity. His distance visual acuity without correction was scored at 5/100; the right eye (RE) improved to 10/100 after the correction of a myopic astigmatism; and the unimproved left eye was scored at 20/100. The eye fundus showed perimacular radial lines without increased separation for the right eye with some microcysts and a macular hole on the left. An examination of the retinal periphery of both the eyes found inferotemporal retinal splitting. The electrophysiological assessment showed a major dysfunction on the electroretinogram.

Keywords: Degeneration, macula, retinoschisis, star
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