LA90.21

ICD-11 MMS 2026-01

Congenital portosystemic shunt

Index terms

  • Congenital portosystemic shunt
  • anomalous portal venous connection
  • anomalous pulmonary venous drainage to hepatic veins
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  • anomaly of portal vein connection
  • portal vein anomaly
  • portal vein deformity
  • Portal vein atresia
  • Portal vein distortion
  • Portal vein hypoplasia
  • Congenital intrahepatic portosystemic shunt
  • Congenital extrahepatic portosystemic shunt
  • Abernethy malformation
  • CEPS - [congenital extrahepatic portosystemic shunt]
  • congenital extrahepatic portosystemic venous shunt
  • Congenital absence of portal vein
  • abernethy malformation type I
  • congenital extrahepatic portosystemic shunt type I
  • portal vein agenesis
  • Congenital extrahepatic portosystemic shunt with hypoplastic intrahepatic portal venous system
  • abernethy malformation type II
  • congenital extrahepatic portosystemic shunt type II

Exclusions from above levels

  • 2E81 – haemangioma and lymphangioma
  • LA13.5 – Congenital retinal aneurysm
  • LA8B.1 – Congenital anomaly of pulmonary arterial tree
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  • LA8C – Congenital anomaly of coronary artery
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LA90.20 – Vein of Galen aneurysm
  • LA90.2Y – Other specified peripheral venous malformations
  • LA90.2Z – Peripheral venous malformations, unspecified

Foundation entity

Congenital portosystemic shunt – http://id.who.int/icd/entity/682625970

Part of: LA90.2: Peripheral venous malformations › LA90: Structural developmental anomalies of the peripheral vascular system › 20: Developmental anomalies