Congenital enlargement of the intrahepatic bile ducts (Caroli disease): causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Congenital dilatations of intrahepatic bile ducts (Caroli's disease) - a rare disease characterized by the presence of congenital segmentary saccular dilations of intrahepatic bile ducts without other histological changes in the liver. Expanded ducts communicate with the main duct system, can be infected and contain stones.
The nature of Karoli's inheritance is not exactly established. Kidneys are usually intact, however, a combination with ectasia of the renal tubules with large cysts is possible.
Symptoms of congenital enlargement of intrahepatic bile ducts (Caroli's disease)
The disease can occur 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 during episodes of cholangitis may be worse. Portal hypertension does not develop.
There is an excessive current of bile, which is strengthened after the administration of secretin, which stimulates duct secretion. Probably, the increased bile current in rest is due to the presence of cysts.
Diagnosis of congenital enlargement of intrahepatic bile ducts (Caroli's disease)
Diagnostics help ultrasound and CT; when contrasting against the background of enlarged intrahepatic bile ducts, branches of the portal vein (a symptom of the "central spot") are revealed. Endoscopic or percutaneous cholangiography allows you to establish a diagnosis. The common bile duct is unchanged, the intrahepatic ducts show areas of saccular expansion alternating with normal ducts. Changes can be localized in one half of the liver. This picture differs from the primary sclerosing cholangitis, in which there are uneven contours and narrowing of the common bile duct and uneven contours and widening of the intrahepatic ducts. With cirrhosis, large bile ducts have smooth contours and envelop the regeneration nodes.
The disease is approximately 7% of cases complicated by cholangiocarcinoma.
Treatment of congenital enlargement of intrahepatic bile ducts (Caroli's disease)
With cholangitis, antibiotics are prescribed; Stones of the common bile duct are removed endoscopically or surgically. With intrahepatic stones, ursodeoxycholic acid is successfully used.
If one half of the liver is affected, resection is possible. The possibility of liver transplantation should be considered, but infection is usually a contraindication to it.
The prognosis is unfavorable, although recurrences of cholangitis can be observed for many years.
Renal failure as the cause of death is uncharacteristic.
Congenital fibrosis of the liver and Caroli's disease
Caroli's disease is often combined with congenital fibrosis of the liver, and this condition is called Caroli syndrome. Both diseases develop due to similar disturbances in the formation of the embryonic duct plate at different levels of the biliary tree. The disease is inherited by autosomal recessive type, manifested by abdominal pain and cholangitis or bleeding from esophageal varices.
In autopsy, neonates describe a combination of signs of congenital liver fibrosis, Caroli's disease and polycystic kidney disease.
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