Kearns-Sayre syndrome, unspecified eye

This code signifies Kearns-Sayre syndrome, a rare mitochondrial disorder characterized by progressive external ophthalmoplegia, pigmentary retinopathy, and cardiac conduction abnormalities. It represents the diagnosis when the specific eye affected by the ophthalmoplegia or retinopathy is not documented.

Use this code when the medical record clearly diagnoses Kearns-Sayre syndrome but does not specify whether the right or left eye is involved. This is appropriate when the ophthalmological findings are generalized or not lateralized in the documentation.

• Do not use this code if the medical record specifies the affected eye (e.g., right eye, left eye).
• Always review documentation for laterality; if specified, use the more precise code (H49.811 or H49.812).
• Consider co-occurring conditions such as cardiac conduction defects (e.g., AV block) and endocrine disorders, and code them separately.