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ICD-10-CM 2027 diagnosis code

H49.81Kearns-Sayre syndrome

Not billable — use a more specific code

H49.81 is a header code, so it can't be reported by itself. Choose one of the 4 more specific codes beneath it.

Looking up by condition? See ICD-10 code for Kearns-Sayre syndrome.

Code unchanged since ICD-10-CM took effect on October 1, 2015. Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data

About H49.81

H49.81 is the ICD-10-CM diagnosis code for Kearns-Sayre syndrome. It belongs to category H49 (paralytic strabismus), block H49-H52 (disorders of ocular muscles, binocular movement, accommodation and refraction) and chapter 7 (diseases of the eye and adnexa). It is a header code, so it can't be reported by itself; one of its 4 subcodes must be used instead.

Within H49.8 (other paralytic strabismus), H49.81 is specifically for Kearns-Sayre syndrome. Related codes cover other paralytic strabismus (H49.88).

To report this condition, pick the subcode that matches the documentation: H49.811 (right eye), H49.812 (left eye), H49.813 (bilateral) and H49.819 (unspecified eye).

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Specific codes under H49.81

Coding notes

Applicable toConditions and synonyms classified to this code
  • Progressive external ophthalmoplegia with pigmentary retinopathy
Code alsoTwo codes may be needed; sequencing depends on circumstances
  • , if applicable, other manifestations, such as:
  • heart block (I45.9)

Notes that apply from higher levels

Instructions written at a parent level also apply to H49.81.

› From H49 Paralytic strabismus
Excludes2
  • internal ophthalmoplegia (H52.51-)
  • internuclear ophthalmoplegia (H51.2-)
  • progressive supranuclear ophthalmoplegia (G23.1)

Broader instructions also apply from H49-H52 Disorders of ocular muscles, binocular movement, accommodation and refraction and Chapter 7: Diseases of the eye and adnexa.

Alphabetic index entries

3 entries

Terms in the official ICD-10-CM index that lead to H49.81.

Codes whose notes reference H49.81

Code history

  1. 2016
  2. 2017
  3. 2018
  4. 2019
  5. 2020
  6. 2021
  7. 2022
  8. 2023
  9. 2024
  10. 2025
  11. 2026
  12. 2027
  • FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
  • No changes since FY2016.

Common questions about H49.81

Is H49.81 billable?

No. H49.81 is a header code. Report one of its more specific subcodes instead.