ICD-10 Code H49.819 – Kearns-Sayre syndrome, unspecified eye (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code H49.819 – Kearns-Sayre syndrome, unspecified eye
What it is
H49.819 identifies Kearns-Sayre syndrome affecting an unspecified eye. Use it when documentation names this mitochondrial disorder but does not specify which eye is involved.
Clinical signs
Clinical features vary; refer to documentation. Kearns-Sayre syndrome is commonly associated with progressive external ophthalmoplegia, ptosis, and other ocular motility problems.
When to use this code
Use this code when the provider documents Kearns-Sayre syndrome and does not identify the right or left eye. It is appropriate for encounter notes, problem lists, or claims that only confirm the diagnosis. If the record specifies one eye, choose the laterality-specific code instead.
Do not use for
Do not use this code for nonspecific ptosis, ophthalmoplegia, or other eye movement disorders without documented Kearns-Sayre syndrome. Check documentation if the eye involved is stated.
Coding tip
Confirm both the syndrome name and laterality before coding, because unspecified eye codes should be used only when the record truly lacks that detail.