2026 ICD-10-CM Diagnosis Code H49.819Kearns-Sayre syndrome, unspecified eye

ICD-10-CM Codes›H00–H59›H49-H52›H49

ICD-10-CM H49.819
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

H49.819 is a billable ICD-10-CM diagnosis code for Kearns-Sayre syndrome, unspecified eye. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). The code is flagged as an unspecified code, since codes identifying laterality exist in the same family. Coders also document this condition as Kearns-Sayre syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Neuro-ophthalmology and Strabismus.

Code Identity

ICD-10-CM Code
H49.819
Billable Status
Yes — Valid for Submission
Code Describes
Kearns-Sayre syndrome, unspecified eye
Short Description
Kearns-Sayre syndrome, unspecified eye
Same as the full description in the CMS dataset.
Parent Code
H49.81
Kearns-Sayre syndrome

Code Classification

ChapterH00–H59Diseases of the eye and adnexa
SectionH49-H52Disorders of ocular muscles, binocular movement, accommodation and refraction
CategoryH49Paralytic strabismus
This CodeH49.819Kearns-Sayre syndrome, unspecified eye

Code EditsBilling

Medicare Code Editor checks that affect claim validity for H49.819.

Unspecified codes
Unspecified codes exist in the ICD-10-CM classification for circumstances when documentation in the medical record does not provide the level of detail needed to support reporting a more specific code. However, in the inpatient setting, there should generally be very limited and rare circumstances for which the laterality (right, left, bilateral) of a condition is unable to be documented and reported. The following pages contain the list of unspecified ICD-10-CM diagnosis codes for which there is a more specific code to identify laterality (right, left, bilateral) within that code family.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Kearns-Sayre syndrome

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR EYE006
Neuro-ophthalmology
Default principal diagnosis: inpatient Yes · outpatient Yes
CCSR EYE007
Strabismus
Default principal diagnosis: inpatient No · outpatient No

Patient EducationClinical

Eye Movement Disorders

When you look at an object, you're using several muscles to move both eyes to focus on it. If you have a problem with the muscles, the eyes don't work properly.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert H49.819 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.87 Dis mitochondrial metab
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About H49.819Overview

Is H49.819 (Kearns-Sayre syndrome) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report Kearns-Sayre syndrome, unspecified eye on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of H49.819?

Under the General Equivalence Mappings, Kearns-Sayre syndrome, unspecified eye converts to ICD-9-CM 277.87 (dis mitochondrial metab). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.