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H49.81

H49.81Kearns-Sayre syndrome

Non-billable headerICD-10-CM

H49.81 is a non-billable header ICD-10-CM code for kearns-sayre syndrome. It cannot be billed on its own: choose one of the more specific child codes below.

Code also

Both codes may be needed to describe the encounter fully. Sequencing depends on the reason for the visit.

  • , if applicable, other manifestations, such as:
  • heart block (I45.9)

Inclusion terms

Alternative wordings a physician may use in the chart for this same code.

  • Progressive external ophthalmoplegia with pigmentary retinopathy

Other codes that can never be billed with H49.81

These codes carry an Excludes1 note pointing at H49.81. The conflict binds both ways, but ICD-10-CM only writes it down on one side — so you would not find this by reading H49.81 alone.

  • E88.4Mitochondrial metabolism disorders
  • G71.3Mitochondrial myopathy, not elsewhere classified
  • H49.4Progressive external ophthalmoplegia

Risk adjustment

H49.81 does not map to an HCC and does not risk-adjust under CMS-HCC V28. That is normal — most ICD-10 codes do not. It still needs to be coded correctly; it just will not move a RAF score.

Code to one of these instead H49.81

4 child codes. Green means billable.

Related codes at this level

Codes that share H49.8. If H49.81 is not quite right, the correct code is usually one of these.

Questions about H49.81

Is H49.81 a billable ICD-10-CM code?
No. H49.81 is a non-billable header code. Code to a higher level of specificity using one of its child codes.
Where does H49.81 sit in the tabular list?
Diseases of the eye and adnexa (H00-H59), in the block Disorders of ocular muscles, binocular movement, accommodation and refraction (H49-H52).
ICD-10-CMFY2026-Apr· effective April 1, 2026

Loaded directly from the CMS/NCHS ICD-10-CM public-domain release — see data sources and our editorial policy. If this page disagrees with the CMS tabular list, this page is wrong. Reference information for professional coders; not medical or billing advice.