2020 ICD-10-CM Diagnosis Code H49.81

Kearns-Sayre syndrome

    2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
  • H49.81 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • The 2020 edition of ICD-10-CM H49.81 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of H49.81 - other international versions of ICD-10 H49.81 may differ.
Applicable To
  • Progressive external ophthalmoplegia with pigmentary retinopathy
Use Additional
Use Additional Help
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. For such conditions the ICD-10-CM has a coding convention that requires the underlying condition be sequenced first followed by the manifestation. Wherever such a combination exists there is a "use additional code" note at the etiology code, and a "code first" note at the manifestation code. These instructional notes indicate the proper sequencing order of the codes, etiology followed by manifestation. In most cases the manifestation codes will have in the code title, "in diseases classified elsewhere." Codes with this title are a component of the etiology/manifestation convention. The code title indicates that it is a manifestation code. "In diseases classified elsewhere" codes are never permitted to be used as first listed or principle diagnosis codes. They must be used in conjunction with an underlying condition code and they must be listed following the underlying condition.
  • code for other manifestation, such as:
  • heart block (
    ICD-10-CM Diagnosis Code I45.9

    Conduction disorder, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Heart block NOS
    • Stokes-Adams syndrome
    I45.9
    )
The following code(s) above H49.81 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to H49.81:
  • H00-H59
    2020 ICD-10-CM Range H00-H59

    Diseases of the eye and adnexa

    Note
    • Use an external cause code following the code for the eye condition, if applicable, to identify the cause of the eye condition
    Type 2 Excludes
    • certain conditions originating in the perinatal period (P04-P96)
    • certain infectious and parasitic diseases (A00-B99)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
    • diabetes mellitus related eye conditions (E09.3-, E10.3-, E11.3-, E13.3-)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • injury (trauma) of eye and orbit (S05.-)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    • syphilis related eye disorders (A50.01, A50.3-, A51.43, A52.71)
    Diseases of the eye and adnexa
  • H49-H52
    2020 ICD-10-CM Range H49-H52

    Disorders of ocular muscles, binocular movement, accommodation and refraction

    Type 2 Excludes
    • nystagmus and other irregular eye movements (H55)
    Disorders of ocular muscles, binocular movement, accommodation and refraction
  • H49
    ICD-10-CM Diagnosis Code H49

    Paralytic strabismus

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 2 Excludes
    • internal ophthalmoplegia (H52.51-)
    • internuclear ophthalmoplegia (H51.2-)
    • progressive supranuclear ophthalmoplegia (G23.1)
    Paralytic strabismus
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.
Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
Code annotations containing back-references to H49.81:
  • Type 1 Excludes: G71.3
    , H49.4
    , E88.4
    ICD-10-CM Diagnosis Code G71.3

    Mitochondrial myopathy, not elsewhere classified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Type 1 Excludes
    • Kearns-Sayre syndrome (H49.81)
    • Leber's disease (H47.21)
    • Leigh's encephalopathy (G31.82)
    • mitochondrial metabolism disorders (E88.4.-)
    • Reye's syndrome (G93.7)
    ICD-10-CM Diagnosis Code H49.4

    Progressive external ophthalmoplegia

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    ICD-10-CM Diagnosis Code E88.4

    Mitochondrial metabolism disorders

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • disorders of pyruvate metabolism (E74.4)
    • Kearns-Sayre syndrome (H49.81)
    • Leber's disease (H47.22)
    • Leigh's encephalopathy (G31.82)
    • Mitochondrial myopathy, NEC (G71.3)
    • Reye's syndrome (G93.7)

Diagnosis Index entries containing back-references to H49.81:
  • Kearns-Sayre syndrome H49.81-
  • Strabismus (congenital) (nonparalytic) H50.9
    ICD-10-CM Diagnosis Code H50.9

    Unspecified strabismus

      2016 2017 2018 2019 2020 Billable/Specific Code
    • paralytic H49.9
      ICD-10-CM Diagnosis Code H49.9

      Unspecified paralytic strabismus

        2016 2017 2018 2019 2020 Billable/Specific Code
      • ophthalmoplegia (external)
        • progressive H49.4-
          ICD-10-CM Diagnosis Code H49.4-

          Progressive external ophthalmoplegia

            2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
          Type 1 Excludes
          • with pigmentary retinopathy H49.81-
      • Kearns-Sayre syndrome H49.81-

ICD-10-CM Codes Adjacent To H49.81
H49.30 …… unspecified eye
H49.31 …… right eye
H49.32 …… left eye
H49.33 …… bilateral
H49.4 Progressive external ophthalmoplegia
H49.40 …… unspecified eye
H49.41 …… right eye
H49.42 …… left eye
H49.43 …… bilateral
H49.8 Other paralytic strabismus
H49.81 Kearns-Sayre syndrome
H49.811 …… right eye
H49.812 …… left eye
H49.813 …… bilateral
H49.819 …… unspecified eye
H49.88 Other paralytic strabismus
H49.881 …… right eye
H49.882 …… left eye
H49.883 …… bilateral
H49.889 …… unspecified eye
H49.9 Unspecified paralytic strabismus

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.