8B22.B

ICD-11 MMS 2026-01

Moyamoya syndrome

Description

A cerebrovascular disease caused by stenotic arteries at the base of the brain in the basal ganglia. Moyamoya, meaning "puff of smoke" refers to the appearance of the anastomotic vessel network formed at the base of the brain distal to the circle of Willis to compensate for the blockage.

Index terms

  • Moyamoya syndrome
  • Moyamoya disease
  • moyamoya
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  • moyamoya disorder
  • progressive intracranial arterial occlusion
  • Secondary moyamoya

Exclusions from above levels

  • 8B25 – Late effects of cerebrovascular disease
  • NA07 – Intracranial injury
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8B22.0 – Dissection of cerebral arteries
  • 8B22.1 – Cerebral venous thrombosis
  • 8B22.2 – Cerebral vasoconstriction syndromes
  • 8B22.3 – Isolated cerebral amyloid angiopathy
  • 8B22.4 – Intracranial vascular malformation
  • 8B22.5 – Cerebral aneurysm, nonruptured
  • 8B22.6 – Familial cerebral saccular aneurysm
  • 8B22.7 – Cerebral arteritis, not elsewhere classified
  • 8B22.8 – Hypertensive encephalopathy
  • 8B22.9 – Migraine-induced stroke
  • 8B22.A – Subclavian steal syndrome
  • 8B22.C – Hereditary cerebrovascular diseases
  • 8B22.Y – Other specified cerebrovascular disease

Foundation entity

Moyamoya syndrome – http://id.who.int/icd/entity/1746892088

Part of: 8B22: Certain specified cerebrovascular diseases › 08: Diseases of the nervous system