2021 ICD-10-CM Diagnosis Code H47.22

Hereditary optic atrophy

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • H47.22 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM H47.22 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of H47.22 - other international versions of ICD-10 H47.22 may differ.
Applicable To
  • Leber's optic atrophy
The following code(s) above H47.22 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 H47.22:
  • H00-H59
    2021 ICD-10-CM Range H00-H59

    Diseases of the eye and adnexa

    • 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
Approximate Synonyms
  • Bilateral hereditary optic atrophy
  • Leber's optic atrophy
  • Lebers optic atrophy
  • Left hereditary optic atrophy
  • Right hereditary optic atrophy
Clinical Information
  • A hereditary disorder caused by mitochondrial mutations, resulting in the degeneration of the retinal ganglion cells and optic atrophy. It is characterized by an acute or subacute loss of central vision. It may initially affect one eye only, but eventually the central loss of vision becomes bilateral.
  • A maternally linked genetic disorder that presents in mid-life as acute or subacute central vision loss leading to central scotoma and blindness. The disease has been associated with missense mutations in the mtdna, in genes for complex i, iii, and iv polypeptides, that can act autonomously or in association with each other to cause the disease. (from online mendelian inheritance in man, http://www.ncbi.nlm.nih.gov/omim/, mim#535000 (april 17, 2001))
  • Hereditary conditions that feature progressive visual loss in association with optic atrophy. Relatively common forms include autosomal dominant optic atrophy (optic atrophy, autosomal dominant) and leber hereditary optic atrophy (optic atrophy, hereditary, leber).
ICD-10-CM H47.22 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 123 Neurological eye disorders

Convert H47.22 to ICD-9-CM

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
  • 2021 (effective 10/1/2020): No change
Code annotations containing back-references to H47.22:
  • Type 1 Excludes: E88.4
    ICD-10-CM Diagnosis Code E88.4

    Mitochondrial metabolism disorders

      2016 2017 2018 2019 2020 2021 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 H47.22:
  • Atrophy, atrophic (of)
    • optic (nerve) H47.20
      ICD-10-CM Diagnosis Code H47.20

      Unspecified optic atrophy

        2016 2017 2018 2019 2020 2021 Billable/Specific Code
      • hereditary H47.22
    • Leber's optic H47.22 (hereditary)
  • Leber's
    • optic atrophy H47.22 (hereditary)
  • Neuroretinopathy, hereditary optic H47.22

ICD-10-CM Codes Adjacent To H47.22
H47.142 …… left eye
H47.143 …… bilateral
H47.149 …… unspecified eye
H47.2 Optic atrophy
H47.20 Unspecified optic atrophy
H47.21 Primary optic atrophy
H47.211 …… right eye
H47.212 …… left eye
H47.213 …… bilateral
H47.219 …… unspecified eye
H47.22 Hereditary optic atrophy
H47.23 Glaucomatous optic atrophy
H47.231 …… right eye
H47.232 …… left eye
H47.233 …… bilateral
H47.239 …… unspecified eye
H47.29 Other optic atrophy
H47.291 …… right eye
H47.292 …… left eye
H47.293 …… bilateral
H47.299 …… unspecified eye

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