ICD-10 Code H49.81 – Kearns-Sayre Syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code H49.81 – Kearns-Sayre syndrome
What it is
Kearns-Sayre syndrome is a rare mitochondrial disorder that affects the eyes and other organs. It is typically characterized by progressive external ophthalmoplegia, onset before age 20, and additional systemic findings.
Clinical signs
Common findings include ptosis, limited eye movements, and pigmentary retinopathy. Clinical features vary; refer to documentation for associated heart block, cerebellar signs, or hearing loss.
When to use this code
Use H49.81 when the provider documents Kearns-Sayre syndrome by name. It also fits confirmed cases with classic ophthalmologic findings and supportive systemic involvement. If the record only notes mitochondrial disease or nonspecific ophthalmoplegia, check documentation before coding.
Do not use for
Do not use this code for isolated ptosis, nonspecific strabismus, or other eye movement disorders without a diagnosis of Kearns-Sayre syndrome. If the syndrome is not confirmed, check documentation.
Coding tip
Code the syndrome only when the diagnosis is explicitly stated or clearly supported in the record.