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:
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:
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.