Short answer · Medically reviewed summary · Last updated: 2023-07-13

Caroli Disease, also known as Caroli syndrome or Caroli's disease, is a rare congenital disorder that affects the bile ducts within the liver. It is characterized by the presence of dilated intrahepatic bile ducts, which can lead to various complications and health issues. Synonyms for Caroli Disease: Caroli Syndrome Caroli's Disease Caroli's Syndrome Intrahepatic Bile Duct Dilatation Intrahepatic Bile Duct Cystic Dilatation The condition is named after the Swiss pathologist Jacques Caroli, who first described it in the 1950s.

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Caroli Disease synonyms

Other names for Caroli Disease: synonyms, acronyms and related terms used by doctors and patients.

Caroli Disease is also known as...


Caroli Disease, also known as Caroli syndrome or Caroli's disease, is a rare congenital disorder that affects the bile ducts within the liver. It is characterized by the presence of dilated intrahepatic bile ducts, which can lead to various complications and health issues.



Synonyms for Caroli Disease:



  • Caroli Syndrome

  • Caroli's Disease

  • Caroli's Syndrome

  • Intrahepatic Bile Duct Dilatation

  • Intrahepatic Bile Duct Cystic Dilatation



The condition is named after the Swiss pathologist Jacques Caroli, who first described it in the 1950s. Caroli Disease is typically present from birth, although symptoms may not manifest until later in life. It is considered a genetic disorder, with both autosomal recessive and autosomal dominant inheritance patterns reported.



Common symptoms and complications associated with Caroli Disease include:



  • Recurrent episodes of abdominal pain

  • Jaundice (yellowing of the skin and eyes)

  • Fever and chills

  • Hepatomegaly (enlarged liver)

  • Cholangitis (inflammation of the bile ducts)

  • Biliary stones

  • Liver abscesses

  • Portal hypertension

  • Cholangiocarcinoma (bile duct cancer)



Diagnosis of Caroli Disease involves imaging tests such as ultrasound, CT scan, or MRI, which can reveal the characteristic dilated bile ducts. Treatment options depend on the severity of symptoms and complications. In some cases, liver transplantation may be necessary.



In conclusion, Caroli Disease, also referred to as Caroli syndrome or Caroli's disease, is a rare congenital disorder characterized by dilated intrahepatic bile ducts. It can lead to various complications and is associated with symptoms such as abdominal pain, jaundice, and hepatomegaly. Early diagnosis and appropriate management are crucial in improving the prognosis for individuals with Caroli Disease.


Author: DiseaseMaps Editorial Team
Reviewed against authoritative medical sources (NIH GARD, Orphanet, OMIM)
Last updated: 2023-07-13
Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
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