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  6. 2024 ICD-10-CM Code H49.81
Version 2024
Non-Billable Code

2024 ICD-10-CM Diagnosis Code H49.81

Kearns-Sayre syndrome

ICD-10-CM Code:
H49.81
ICD-10 Code for:
Kearns-Sayre syndrome
Is Billable?
Not Valid for Submission
Code Navigator:

Code Classification

  • Diseases of the eye and adnexa
    (H00–H59)
    • Disorders of ocular muscles, binocular movement, accommodation and refraction
      (H49-H52)
      • Paralytic strabismus
        (H49)

H49.81 is a non-specific and non-billable diagnosis code code, consider using a code with a higher level of specificity for a diagnosis of kearns-sayre syndrome. The code is not specific and is NOT valid for the year 2024 for the submission of HIPAA-covered transactions. Category or Header define the heading of a category of codes that may be further subdivided by the use of 4th, 5th, 6th or 7th characters.

Specific Coding Applicable to Kearns-Sayre syndrome

Non-specific codes like H49.81 require more digits to indicate the appropriate level of specificity. Consider using any of the following ICD-10-CM codes with a higher level of specificity when coding for kearns-sayre syndrome:

  • Use H49.811 for Kearns-Sayre syndrome, right eye - BILLABLE CODE

  • Use H49.812 for Kearns-Sayre syndrome, left eye - BILLABLE CODE

  • Use H49.813 for Kearns-Sayre syndrome, bilateral - BILLABLE CODE

  • Use H49.819 for Kearns-Sayre syndrome, unspecified eye - BILLABLE CODE

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Progressive external ophthalmoplegia with pigmentary retinopathy

Code Also

Code Also
A "code also" note instructs that two codes may be required to fully describe a condition, but this note does not provide sequencing direction.
  • , if applicable, other manifestations, such as:
  • heart block I45.9

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Patient Education


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.

There are many kinds of eye movement disorders. Two common ones are:

  • Strabismus - a disorder in which the two eyes don't line up in the same direction. This results in "crossed eyes" or "walleye."
  • Nystagmus - fast, uncontrollable movements of the eyes, sometimes called "dancing eyes"

Some eye movement disorders are present at birth. Others develop over time and may be associated with other problems, such as injuries. Treatments include glasses, patches, eye muscle exercises, and surgery. There is no cure for some kinds of eye movement disorders, such as most kinds of nystagmus.


[Learn More in MedlinePlus]

Kearns-Sayre syndrome

Kearns-Sayre syndrome is a condition that affects many parts of the body, especially the eyes. The features of Kearns-Sayre syndrome usually appear before age 20, and the condition is diagnosed by a few characteristic signs and symptoms. People with Kearns-Sayre syndrome have progressive external ophthalmoplegia, which is weakness or paralysis of the eye muscles that impairs eye movement and causes drooping eyelids (ptosis). Affected individuals also have an eye condition called pigmentary retinopathy, which results from breakdown (degeneration) of the light-sensing tissue at the back of the eye (the retina) that gives it a speckled and streaked appearance. The retinopathy may cause loss of vision. In addition, people with Kearns-Sayre syndrome have at least one of the following signs or symptoms: abnormalities of the electrical signals that control the heartbeat (cardiac conduction defects), problems with coordination and balance that cause unsteadiness while walking (ataxia), or abnormally high levels of protein in the fluid that surrounds and protects the brain and spinal cord (the cerebrospinal fluid or CSF).

People with Kearns-Sayre syndrome may also experience muscle weakness in their limbs, deafness, kidney problems, or a deterioration of cognitive functions (dementia). Affected individuals often have short stature. In addition, diabetes mellitus is occasionally seen in people with Kearns-Sayre syndrome.

When the muscle cells of affected individuals are stained and viewed under a microscope, these cells usually appear abnormal. The abnormal muscle cells contain an excess of structures called mitochondria and are known as ragged-red fibers.

A related condition called ophthalmoplegia-plus may be diagnosed if an individual has many of the signs and symptoms of Kearns-Sayre syndrome but not all the criteria are met.


[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.