Caroli Disease

Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.

Also Known As:

Caroli's Disease; Caroli's Syndrome; Caroli Syndrome; Carolis Disease; Carolis Syndrome; Disease, Caroli; Disease, Caroli's; Syndrome, Caroli's

Digestive System Diseases: 1068
- Biliary Tract Diseases: 470
  - Bile Duct Diseases: 96
    - Choledochal Cyst: 609
      - Caroli Disease: 154
- Digestive System Abnormalities
  - Choledochal Cyst: 609
    - Caroli Disease: 154
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 960
- Congenital Abnormalities: 26404
  - Multiple Abnormalities: 213
    - Ciliopathies: 632
      - Caroli Disease: 154
  - Digestive System Abnormalities
    - Choledochal Cyst: 609
      - Caroli Disease: 154
- Inborn Genetic Diseases: 12342
  - Ciliopathies: 632
    - Caroli Disease: 154

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Disease

Drug/Important Bio-Agent (IBA)