H49.81

Applicable To

• Progressive external ophthalmoplegia with pigmentary retinopathy

Clinical Information

• A mitochondrial disorder featuring the triad of chronic progressive external ophthalmoplegia, cardiomyopathy cardiomyopathies) with conduction block heart block), and retinitis pigmentosa. Disease onset is in the first or second decade. Elevated csf protein, sensorineural deafness, seizures, and pyramidal signs may also be present. Ragged-red fibers are found on muscle biopsy. Adams et al., Principles of Neurology, 6th ed, p984)

• A rare mitochondrial myopathy characterized by a progressive limitation of eye movements, leading to immobility and eye drop. It may be associated with muscle weakness, heart block, hearing loss, ataxia and short stature.

Details

• H49.81 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.

• The 2023 edition of ICD-10-CM H49.81 became effective on October 1, 2022.

• This is the American ICD-10-CM version of H49.81 – other international versions of ICD-10 H49.81 may differ.