9C40.8

ICD-11 MMS 2026-01

Hereditary optic neuropathy

Description

Optic nerve disorders caused by genetic abnormalities

Index terms

  • Hereditary optic neuropathy
  • Autosomal-Dominant optic atrophy
  • Dominant optic atrophy
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  • Childhood-onset autosomal dominant optic atrophy
  • Autosomal dominant optic atrophy, Kjer type
  • Autosomal dominant optic atrophy, classic type
  • Kjer disease
  • Autosomal dominant optic atrophy plus syndrome
  • Hereditary Optic Neuropathy associated with hereditary polyneuropathies
  • Peripheral neuropathy and optic atrophy
  • Autosomal dominant optic atrophy and cataract
  • Autosomal-recessive optic atrophy
  • Autosomal recessive optic atrophy type 6
  • Autosomal recessive optic atrophy type 7
  • Sex-linked optic atrophy

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes
  • 8A21.0 – Posterior cortical atrophy

Sibling codes

  • 9C40.0 – Infectious optic neuropathy
  • 9C40.1 – Optic neuritis
  • 9C40.2 – Neuroretinitis
  • 9C40.3 – Perineuritis of optic nerve
  • 9C40.4 – Ischaemic optic neuropathy
  • 9C40.5 – Compressive optic neuropathy
  • 9C40.6 – Infiltrative optic neuropathy
  • 9C40.7 – Traumatic optic neuropathy
  • 9C40.9 – Glaucomatous optic neuropathy
  • 9C40.A – Optic disc swelling
  • 9C40.B – Optic atrophy
  • 9C40.Y – Other specified disorder of the optic nerve
  • 9C40.Z – Disorder of the optic nerve, unspecified

Foundation entity

Hereditary optic neuropathy – http://id.who.int/icd/entity/2452831

Allowed postcoordination axes

Part of: 9C40: Disorder of the optic nerve › 09: Diseases of the visual system