9C40.B

ICD-11 MMS 2026-01

Optic atrophy

Description

Optic atrophies (OA) refer to a specific group of hereditary optic neuropathies in which the cause of the optic nerve dysfunction is inherited either in an autosomal dominant or autosomal recessive pattern. Autosomal dominant optic atrophy (ADOA), type Kjer, is the most common OA, whereas autosomal recessive optic atrophy (AROA) is a rare form.

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

Child codes

  • 9C40.B0 – Congenital optic atrophy
  • 9C40.B1 – Acquired optic atrophy
  • 9C40.BZ – Optic atrophy, unspecified

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
  • 9C40.8 – Hereditary optic neuropathy
  • 9C40.9 – Glaucomatous optic neuropathy
  • 9C40.A – Optic disc swelling
  • 9C40.Y – Other specified disorder of the optic nerve
  • 9C40.Z – Disorder of the optic nerve, unspecified

Foundation entity

Optic atrophy – http://id.who.int/icd/entity/568505454

Allowed postcoordination axes

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