Contributor: Gordon K. Klintworth
Gyrate atrophy of the retina and choroid (gyrate atrophy of the choroid and retina, hyperornithinemia, chorioretinal gyrate atrophy, gyrate choroiditis) is an inherited progressive degenerative retinopathy with an autosomal recessive mode of inheritance. The disorder is associated with an elevated ornithine in the blood and urine and a reduction or absence of the mitochondrial matrix enzyme ornithine aminotransferase. The clinical picture of gyrate atrophy of the choroid and retina can occur in the absence of hyperornithinemia.