Caroli's disease. Report of 5 cases and review of literature.

In 1958 Caroli was the first to define exactly in literature the congenital dilatations of the intrahepatic bile ducts, concerning a segment, a lobe, or both lobes of the Liver. Later Todani involved it in his classification of the bile duct cysts as type V. Caroli's disease (CD) is quite a rare entity (about 150 cases reported in literature). In CD, stenosis and dilatation cause initially biliary stasis, leading to intrahepatic biliary lithiasis and secondary infection. The spontaneous course of CD is dominated by biliary infection: cholangitis (cholangiohepatitis), septicemia and intrahepatic and subphrenic abscesses. That is why the disease must be recognized before serious complications develop. The authors report about 5 cases of Caroli's disease--2 women and 3 men. Diffuse spread in one patient (25-year-old man) and in four--left lobe involved predominantly. Most common signs and symptoms were: fever, cholangitis, upper quadrant abdominal pain, hepatosplenomegaly, obstructive jaundice, Charcot's triad (in two). Importance for the exact diagnosis involves the use of ultrasonography (US), computer tomography (CT) scan, endoscopic cholangiopancreatography (ERCP), intraoperative cholangiography and cholangioscopy, allowing early diagnosis and therefore a better therapeutic and surgical approach.