Clinical characteristics of Caroli’s disease

Abstract

Caroli's disease is a rare congenital condition characterized by non-obstructive saccular or fusiform dilatation of larger intrahepatic bile ducts. Cholangitis, liver cirrhosis, and cholangiocarcinoma are its potential complications. The diagnosis of Caroli's disease depends on demonstrating that the cystic lesions are in continuity with the biliary tree which can be showed by ultrasonography, computerized tomography, endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography or magnetic resonance cholangiopancreatography. Treatment of Caroli's disease relies on the location of the biliary abnormalities. While localized forms confined to one lobe can be treated with surgery, liver transplantation is the only effective modality for diffuse forms. Although a rare disorder; Caroli's disease should always be considered in the differential diagnosis of chronic cholestasis of unknown cause.

Caroli Disease: A Case Report Observed at the Cocody Teaching Hospital of Abidjan (Cote D’Ivoire)

“…Therefore, this stasis of bile will favor infection syndromes which are the clinical manifestations of the disease. Like for our case, this typical clinical feature was described by different authors [4] [7]. At the end stage of the disease, cirrhosis including an hepatomegaly, an ascite and lower limbs edema could be associated like in the case of our patient [1] [13].…”

“…At the end stage of the disease, cirrhosis including an hepatomegaly, an ascite and lower limbs edema could be associated like in the case of our patient [1] [13]. The diagnostic of this affection is based on radiologic exams [5] [7]. It could be evocated during an abdominal ultrasonography which shows cystic dilations inside the liver parenchyma.…”

“…When she is associated with a congenital hepatic fibrosis, she is called Caroli syndrome. In this case, clinical manifestations are those of congenital hepatic fibrosis including collateral venous circulation, esophageal varices and a spleno-megaly [3] [7]. However, there are some isolated cases [3] [7].…”

“…In this case, clinical manifestations are those of congenital hepatic fibrosis including collateral venous circulation, esophageal varices and a spleno-megaly [3] [7]. However, there are some isolated cases [3] [7]. The prognosis factors depend on its complications: cholangitis, cirrhosis and cholangiocarcinoma [1] [4].…”

An exceptional case of spontaneous fistulization of an intrahepatic biliary cyst in to the colon, operated Tikur Anbessa Specialized Hospital, Ethiopia - a case report

“…This condition (presence of type V biliary cyst) is called Caroli's disease and when associated with congenital hepatic fibrosis it is termed Caroli's Syndrome. 18,19 . In our case, the presence of a single bile containing intrahepatic cyst in the absence of extrahepatic biliary tree involvement and in the absence features of hepatic fibrosis(even though not biopsy proven) suggests simple Caroli's disease.Although present from birth, Caroli's disease usually remains asymptomatic during the first 20 years, and may also remain so throughout life 18 .…”