9B78.0

ICD-11 MMS 2026-01

Retinal vasculopathy and cerebral leukodystrophy

Description

Retinal vasculopathy and cerebral leukodystrophy is an inherited group of small vessel diseases comprised of cerebroretinal vasculopathy, hereditary vascular retinopathy and hereditary endotheliopathy with retinopathy, nephropathy and stroke (HERNS); all exhibiting progressive visual impairment as well as variable cerebral dysfunction.

Index terms

  • Retinal vasculopathy and cerebral leukodystrophy
  • Cerebroretinal vasculopathy
  • Hereditary vascular retinopathy

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

  • 9B78.1 – Background retinopathy and retinal vascular changes
  • 9B78.2 – Other proliferative retinopathy
  • 9B78.3 – Degeneration of macula or posterior pole
  • 9B78.4 – Peripheral retinal degeneration
  • 9B78.5 – Retinal haemorrhage
  • 9B78.6 – Separation of retinal layers
  • 9B78.7 – Retinal oedema
  • 9B78.8 – Retinal ischaemia
  • 9B78.9 – Retinal atrophy

Foundation entity

Retinal vasculopathy and cerebral leukodystrophy – http://id.who.int/icd/entity/554838792

Allowed postcoordination axes

Part of: 9B78: Certain specified retinal disorders › 09: Diseases of the visual system