Abstract

 


Gyrate atrophy of the choroid and retina is a rare chorioretinal dystrophy that begins in childhood and leads to blindless in the fourth to seventh decades of life. The disease is traited in an autosomal recessive manner. The primary defect is deficiency of ornithine delta aminotransferase and causes an accumulation of ornithine. Ocular findings include chorioretinal atrophy, axial myopia, cataract and visual field loss. We report herein two cases of gyrate atrophy in one family. [Journal of Turgut Ozal Medical Center 1996;3(3) :226-229]

Key Words: Gyrate atrophy, hyperornithinemia