Caroli's Disease

Caroli's disease is a rare disease characterized by dilation of the bile ducts within the liver. It is a congenital disease, therefore present from birth, but not familial (that is, not hereditary) that affects only some sections of the biliary tree, without causing other anomalies. Dilated bile ducts can lead to infection or promote stone formation. In 3/4 of the cases it affects the male sex.


Caroli's disease is usually diagnosed in childhood or adolescence, although it can appear at any age. The most common symptoms are abdominal pain, enlarged liver and fever due to bile duct infection (cholangitis), during which jaundice (yellowing of the skin and eyes) may also occur.


In general, a liver ultrasound is enough to initiate a diagnostic suspicion, which must then be confirmed with a more specific examination, such as a CT (Computerized Tomography) with contrast medium. Cholangiography, performed retrograde during endoscopy or percutaneous transhepatic, can definitively confirm the diagnosis. These methods show bag-like dilations of the intrahepatic ducts, alternating with normal tracts; the common bile duct is usually normal, unlike in primary sclerosing cholangitis. Approximately 7% of patients with Caroli's disease develop intrahepatic cholangiocarcinoma throughout their lives, so strict radiological monitoring is important to detect early signs of malignant degeneration.


antibiotic therapy should be given during episodes of cholangitis. To remove the stones, both endoscopic and surgical drainage of the common bile duct may be necessary , or extracorporeal lithotripsy , a non-invasive method that applies shock waves and fragments the stones, may be used . Liver resection may be necessary when a lobe of the liver is involved. In rare cases, liver transplantation may be indicated , although recurrent infections are often a contraindication.