2027 ICD-10-CM Diagnosis Code H47.22Hereditary optic atrophy

ICD-10-CM Codes›H00–H59›H46-H47›H47

ICD-10-CM H47.22
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

H47.22 is a billable ICD-10-CM diagnosis code for hereditary optic atrophy. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 123. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Neuro-ophthalmology.

Code Identity

ICD-10-CM Code
H47.22
Billable Status
Yes — Valid for Submission
Code Describes
Hereditary optic atrophy
Short Description
Hereditary optic atrophy
Same as the full description in the CMS dataset.
Parent Code
H47.2
Optic atrophy

Code Classification

ChapterH00–H59Diseases of the eye and adnexa
SectionH46-H47Disorders of optic nerve and visual pathways
CategoryH47Other disorders of optic [2nd] nerve and visual pathways
This CodeH47.22Hereditary optic atrophy

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Auditory neuropathy, optic atrophy syndrome
  • Autosomal dominant optic atrophy and cataract
  • Autosomal dominant optic atrophy and peripheral neuropathy syndrome
  • Autosomal dominant optic atrophy classic form
  • Autosomal dominant optic atrophy plus syndrome
  • Autosomal recessive bilateral optic atrophy
  • Autosomal recessive isolated optic atrophy
  • Autosomal recessive optic atrophy type 6
  • Autosomal recessive optic atrophy type 7
  • Autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome
  • Behr syndrome
  • Bilateral optic atrophy of eyes
  • Bilateral primary optic atrophy
  • CAMOS syndrome
  • Cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndrome
  • Childhood-onset autosomal dominant optic atrophy
  • Chronic axonal neuropathy
  • Congenital atrophy of optic nerve
  • Diffuse atrophy of cerebrum
  • Dominant hereditary optic atrophy
  • Early-onset progressive diffuse brain atrophy, microcephaly, muscle weakness, optic atrophy syndrome
  • Early-onset X-linked optic atrophy
  • False anodontia
  • GAPO syndrome
  • Generalized dystonia
  • Genetic anomaly of leukocyte
  • Global brain atrophy
  • Hereditary bilateral optic atrophy
  • Hereditary left optic atrophy
  • Hereditary motor and sensory neuropathy with optic atrophy
  • Hereditary optic atrophy
  • Hereditary right optic atrophy
  • Infantile-onset axonal motor and sensory neuropathy, optic atrophy, neurodegenerative syndrome
  • Leber plus disease
  • Leber's optic atrophy
  • Left primary optic atrophy
  • MEPAN syndrome
  • Optic atrophy, ataxia, peripheral neuropathy, global developmental delay syndrome
  • Optic atrophy, intellectual disability syndrome
  • Pelger-Huët anomaly
  • Pelger-Huët cell
  • Right primary optic atrophy
  • Second cranial nerve finding
  • Severe X-linked intellectual disability Gustavson type
  • Short stature, optic nerve atrophy, Pelger-Huët anomaly syndrome
  • Spastic paraplegia, optic atrophy, neuropathy syndrome
  • SPOAN and SPOAN-related disorder
  • Wolfram syndrome
  • Wolfram syndrome type 1
  • Wolfram syndrome type 2
  • X-linked optic atrophy

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to H47.22: its own notes plus those printed at Chapter 7. A note printed at a category, block or chapter applies to every code under it.

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

  • Leber's optic atrophy

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Chapter 7 (H00-H59) Diseases of the eye and adnexa applies to 2,677 codes
  • 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)

Notes

General instructions on how these codes are used.

From Chapter 7 (H00-H59) Diseases of the eye and adnexa applies to 2,677 codes
  • Use an external cause code following the code for the eye condition, if applicable, to identify the cause of the eye condition

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name H47.22, its category, or a range that includes it.

Excludes1 1

These codes carry an Excludes1 note naming H47.22: they are not reported together with it, unless the two conditions are unrelated.

  • E88.4 Mitochondrial metabolism disorders
    Leber's disease (H47.22)

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

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

CCSR EYE006
Neuro-ophthalmology
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Optic Atrophies, Hereditary

    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).

Patient EducationClinical

Optic Nerve Disorders

The optic nerve is a bundle of more than 1 million nerve fibers that carry visual messages. You have one connecting the back of each eye (your retina) to your brain. Damage to an optic nerve can cause vision loss. The type of vision loss and how severe it is depends on where the damage occurs. It may affect one or both eyes.

Read the full article at MedlinePlus

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

Convert H47.22 to ICD-9-CMHistory

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

ICD-9-CM
377.16 Hereditary optic atrophy
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About H47.22Overview

What is the ICD-10 code for hereditary optic atrophy?

The ICD-10-CM code for hereditary optic atrophy is H47.22 (sometimes written as H4722). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is H47.22 (Optic atrophy) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hereditary optic atrophy on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does H47.22 group to?

When hereditary optic atrophy is the principal diagnosis on an inpatient stay, it groups to MS-DRG 123 (neurological Eye Disorders), which carries a relative weight of 0.8218. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of H47.22?

Under the General Equivalence Mappings, hereditary optic atrophy converts to ICD-9-CM 377.16 (hereditary optic atrophy). The mapping is a direct match.