ICD-10 Code H49.811 – Kearns-Sayre syndrome, right eye (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Kearns-Sayre syndrome, right eye is H49.811.
2026 ICD-10-CM Diagnosis Code H49.811 – Kearns-Sayre syndrome, right eye

What it is

H49.811 identifies Kearns-Sayre syndrome affecting the right eye. Use it when documentation specifies this mitochondrial ophthalmoplegia is limited to, or coded for, the right eye.

Clinical signs

Typical findings include progressive external ophthalmoplegia and ptosis, with eye movement limitation. Clinical features vary; refer to documentation for laterality and any associated systemic manifestations.

When to use this code

Use this code when the provider documents Kearns-Sayre syndrome in the right eye, or right-sided ocular involvement in a confirmed case. It is appropriate for diagnoses recorded in ophthalmology, neurology, or genetics notes. If laterality is not stated, check documentation before coding.

Do not use for

Do not use it for nonspecific ptosis, generic ophthalmoplegia, or Kearns-Sayre syndrome without right-eye specificity. If the record does not clearly identify the right eye, do not assume laterality.

Coding tip

Verify both the syndrome name and laterality in the source note before assigning H49.811.

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