ICD-10 Code H49.4 – Progressive external ophthalmoplegia (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code H49.4 – Progressive external ophthalmoplegia
What it is
H49.4 identifies progressive external ophthalmoplegia, a disorder marked by slowly worsening weakness of the eye muscles. It affects eye movement, especially upward, downward, and sideways gaze.
Clinical signs
Typical findings include ptosis and limited extraocular movement on exam. Clinical features vary; refer to documentation, especially when the note describes bilateral involvement or a progressive course.
When to use this code
Use H49.4 when the provider documents progressive external ophthalmoplegia as the diagnosis. Code it for outpatient visits, specialty evaluations, or problem-list entries when the condition is confirmed or clearly stated.
If the record only mentions eye movement weakness without this specific diagnosis, check documentation before assigning the code. Use the documented term, not a presumed cause or a broader neurologic disorder.
Do not use for
Do not use this code for temporary eye movement limitation from trauma, inflammation, or nerve palsy unless progressive external ophthalmoplegia is specifically documented. Check documentation for congenital or other distinct ophthalmologic disorders.
Coding tip
Code the exact diagnosis wording and verify whether the provider documented progressive, external, or bilateral involvement.