DB98.5

ICD-11 MMS 2026-01

Budd-Chiari syndrome

Description

Budd-Chiari syndrome (BCS) is caused by obstruction of hepatic venous outflow involving either the hepatic veins or the terminal segment of the inferior vena cava and leading to hepatic congestion and ischemic necrosis. Severity depends on the speed of onset and extent of the obstruction. Obstructions are generally caused by thrombosis in primary BCS, while secondary BCS results from tumour invasion into the lumen or compression of the vein by an expansive lesion. The principle manifestations of BCS are ascites leading to undernutrition and renal insufficiency, gastrointestinal haemorrhage due to portal hypertension, and hepatic insufficiency resulting in encephalopathy and severe infections. Asymptomatic forms have also been reported.

Index terms

  • Budd-Chiari syndrome
  • Chiari syndrome
  • hepatic vein block
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  • hepatic vein obstruction
  • hepatic venous block
  • obstruction of hepatic veins
  • Budd-Chiari syndrome without inferior vena cava obstruction
  • Budd-Chiari syndrome with inferior vena cava obstruction
  • Hepatic vein and inferior vena cava obstruction
  • Budd-Chiari syndrome with hepatic vein thrombosis
  • Budd-Chiari syndrome with hepatic thrombosis
  • hepatic venous thrombosis
  • liver thrombosis
  • Budd-Chiari syndrome with hepatic vein embolism
  • Budd-Chiari syndrome with hepatic embolism
  • hepatic venous embolism

Exclusions from above levels

  • ME10.1 – Unspecified jaundice
  • 01 – Certain infectious or parasitic diseases
  • 02 – Neoplasms
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  • 05 – Endocrine, nutritional or metabolic diseases
  • 06 – Mental, behavioural or neurodevelopmental disorders
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • DB98.0 – Infarction of liver
  • DB98.1 – Peliosis hepatis
  • DB98.2 – Nodular regenerative hyperplasia of liver
  • DB98.3 – Portal vein thrombosis
  • DB98.4 – Splenic vein thrombosis
  • DB98.6 – Hepatic veno-occlusive disease
  • DB98.7 – Portal hypertension
  • DB98.8 – Passive congestion of liver
  • DB98.9 – Hepatic artery aneurysm
  • DB98.A – Hepatic haemorrhage
  • DB98.B – Ischaemia reperfusion injury of liver
  • DB98.Y – Other specified vascular disorders of the liver
  • DB98.Z – Vascular disorders of the liver, unspecified

Foundation entity

Budd-Chiari syndrome – http://id.who.int/icd/entity/1300118676

Allowed postcoordination axes

Part of: DB98: Vascular disorders of the liver › 13: Diseases of the digestive system