All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Congenital dilation of intrahepatic bile ducts (Caroli's disease): causes, symptoms, diagnosis, treatment
Medical expert of the article
Last reviewed: 07.07.2025
Congenital dilatations of the intrahepatic bile ducts (Caroli disease) - this rare disorder is characterized by the presence of congenital segmental saccular dilatations of the intrahepatic bile ducts without other histological changes in the liver. The dilated ducts communicate with the main duct system, can become infected and contain stones.
The inheritance pattern of Caroli disease is not precisely established. The kidneys are usually intact, but a combination with renal tubular ectasia with large cysts is possible.
Symptoms of congenital dilation of the intrahepatic bile ducts (Caroli disease)
The disease can manifest itself at any age, but more often in children and young people, in the form of abdominal pain, hepatomegaly, fever in the presence of gram-negative septicemia. Approximately 75% of patients are men.
Jaundice is absent or mild, but may increase during episodes of cholangitis. Portal hypertension does not develop.
Excessive bile flow is observed, which increases after the introduction of secretin, which stimulates ductal secretion. The increased bile flow at rest is probably due to the presence of cysts.
Diagnosis of congenital dilation of the intrahepatic bile ducts (Caroli disease)
Ultrasound and CT help in diagnosis; with contrast, branches of the portal vein are revealed against the background of dilated intrahepatic bile ducts (symptom of the "central spot"). Endoscopic or percutaneous cholangiography allows establishing the diagnosis. The common bile duct is unchanged, areas of saccular dilation are expressed in the intrahepatic ducts, alternating with normal ducts. Changes can be localized in one half of the liver. This picture differs from primary sclerosing cholangitis, in which uneven contours and narrowing of the common bile duct and uneven contours and dilation of the intrahepatic ducts are noted. In cirrhosis, large bile ducts have smooth contours and go around the regeneration nodes.
The disease is complicated by cholangiocarcinoma in approximately 7% of cases.
Treatment of congenital dilation of the intrahepatic bile ducts (Caroli disease)
In case of cholangitis, antibiotics are prescribed; common bile duct stones are removed endoscopically or surgically. In case of intrahepatic stones, ursodeoxycholic acid is successfully used.
If one side of the liver is affected, resection may be possible. Liver transplantation should be considered, but infection is usually a contraindication.
The prognosis is poor, although relapses of cholangitis may occur for many years.
Renal failure as a cause of death is uncommon.
Congenital liver fibrosis and Caroli disease
Caroli disease is often associated with congenital liver fibrosis, a condition known as Caroli syndrome. Both diseases result from similar abnormalities in the formation of the embryonic ductal plate at different levels of the biliary tree. The disease is inherited in an autosomal recessive manner and presents with abdominal pain and cholangitis or bleeding from esophageal varices.
Autopsy studies in newborns have described a combination of features of congenital liver fibrosis, Caroli disease, and polycystic kidney disease.
[ What's bothering you?
What do need to examine?