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Caroli disease (kuh-ROH-leez duh-zeez) is a rare inherited disorder that dilates the bile ducts from the liver. There are two types of Caroli disease, the most common being the simple, or isolated case where the bile ducts are widened by ectasia. The second, more complex, case is commonly known as Caroli Syndrome. This complex form is also linked with portal hypertension and congenital hepatic fibrosis. [1] The differences between the causes of the two cases have not yet been discovered. Caroli disease is also associated with liver failure and polycystic kidney disease. The disease affects about 1 in 1,000,000 people, with more reported cases of Caroli syndrome than of Caroli disease. [2]
Caroli disease also is known as communicating cavernous ectasia, or congenital cystic dilatation of the intrahepatic biliary tree. Caroli disease is distinct from other diseases that cause ducal dilatation caused by obstruction, in that it is not one of the many choledochal cyst derivatives. [1]
Jacques Caroli, a gastroenterologist, first described a rare congenital condition in 1958. [3][4] He described it as “nonobstructive saccular or fusiform multi-focal segmental dilatation of the intra-hepatic bile ducts”; basically, he observed cavernous ectasia in the biliary tree causing a chronic, often life-threatening hepatobiliary disease. [5] Caroli, born in France in 1902, learned and practiced medicine in Angers. After World War II he was chief of service for 30 years at Saint-Antoine in Paris. Before dying in 1979, he was honored with the rank of commander in the Legion of Honour in 1976.[4]
The first symptoms typically include fever, intermittent abdominal pain, and hepatomegaly. Occasionally jaundice occurs. [6] Caroli disease usually occurs in the presence of other diseases, such as autosomal recessive polycystic kidney disease, cholangitis, gallstones, bilary abscess, septicemia, liver cirrhosis, renal failure, and cholangiocarcinoma (7% affected).[1] People with Caroli disease are 100 times more at risk for cholangiocarcinoma than the general population.[6] After recognizing symptoms of related diseases, Caroli disease can be diagnosed.
Modern imaging techniques allow the diagnosis to be made more easily and without invasive imaging of the biliary tree.[7] Commonly the disease is limited to the left lobe of the liver. Images taken by CT-scan, X-ray, or MRI will show enlarged intrahepatic (in the liver) bile ducts due to ectasia. Using an ultrasound, tubular dilation of the bile ducts can be seen. On a CT-Scan, Caroli disease can be observed by noting the many fluid-filled, tubular structures extending to the liver.[6] A high contrast CT must be used to distinguish the difference between stones and widened ducts. Bowel gas and digestive habits make it difficult to obtain a clear sonogram, therefore, a CT scan is a good substitution. When the intrahepatic bile duct wall has protrusions, it is clearly seen as central dots or a linear streak.[8] Caroli disease is commonly diagnosed after this “central dot sign” is detected on a CT scan or ultrasound.[8] However, cholangiography is the best, and final, approach to show the enlarged bile ducts as a result of Caroli disease.
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