Gyrate atrophy, choroid

This code describes gyrate atrophy of the choroid, a rare, inherited metabolic disorder characterized by progressive chorioretinal degeneration. It results from a deficiency in the enzyme ornithine aminotransferase, leading to elevated ornithine levels and subsequent atrophy of the choroid and retina. Patients experience progressive vision loss, typically starting with night blindness and peripheral field constriction.

Use this code when documentation confirms a diagnosis of gyrate atrophy affecting the choroid. This diagnosis is typically established through ophthalmological examination revealing characteristic chorioretinal lesions, often accompanied by biochemical testing showing elevated plasma ornithine levels. It is appropriate for both initial diagnosis and subsequent follow-up visits for this specific condition.

• Ensure documentation specifies "gyrate atrophy" and clearly links it to the choroid.
• Consider co-reporting with codes for associated systemic manifestations, if present and documented (e.g., hyperornithinemia).
• Do not confuse with other forms of chorioretinal atrophy unless gyrate atrophy is explicitly stated.