ICD-10 Code H49.813 – Kearns-Sayre syndrome, bilateral (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code H49.813 – Kearns-Sayre syndrome, bilateral
What it is
H49.813 identifies bilateral Kearns-Sayre syndrome, a rare disorder involving both eyes. Use it when documentation specifies this syndrome and bilateral ocular involvement.
Clinical signs
Typical findings include progressive external ophthalmoplegia and ptosis, with eye movement limitation in both eyes. Clinical features vary; refer to documentation.
When to use this code
Use this code when the provider documents Kearns-Sayre syndrome affecting both eyes, or bilateral ophthalmoplegia linked to this diagnosis. It may appear in neurology, ophthalmology, or genetic disorder notes. Confirm laterality and syndrome wording in the record.
Do not use for
Do not use it for unspecified or unilateral Kearns-Sayre syndrome, or for other causes of ptosis or ophthalmoplegia. Check documentation.
Coding tip
Code the laterality exactly as documented; if bilateral is not stated, do not assume it.