Bilateral Juvenile Retinoschisis in four Brothers of a Family.

Noh, Byung Bae; Lee, Sang Joon; Lee, Ho Kyung

Case Report

Journal of the Korean Ophthalmological Society 1993;34(10):1060-1067.
Published online October 1, 1993.

Bilateral Juvenile Retinoschisis in four Brothers of a Family.

Byung Bae Noh, Sang Joon Lee, Ho Kyung Lee

¹Department of Ophthalmoloy, Capital Armed Forces General Hospital, Seoul, Korea.
²Department of Ophthalmology, Inje University, College of Medicine, Paik Hospital, Seoul, Korea.

4 형제의 양안에 발생한 연소기 망막분리증

노병배(Byung Bae Noh),이상준(Sang Joon Lee),이호경(Ho Kyung Lee)

Abstract

Juvenile retinoschisis is a vitreoretinal dystrophy with X-linked recessive mode of transmission that shows microcystic degeneration of the macula associated with splitting of the sensory retina, predominantly within the nerve fiber layer. We experienced X-linked juvenile retinoschisis, in four borthers within a family in which were the onset of visual disturbance between second decades and third decades, and all showed maculopathy, RPE atrophy, vitreous veils extended to vitreous, partial optic atrophy, specific electroretinographic findings.

Key Words: X-linked recessive;retinoschisis;Vitreoretinal dystrophy;Vitreous veils