Caroli disease is a nonobstructive dilatation of the intrahepatic bile ducts. This is a rare congenital disorder that classically causes saccular ductal dilatation, which usually is segmental. Caroli disease is associated with recurrent bacterial cholangitis and stone formation.
Caroli's disease is a congenital disease of cystic or saccular dilatation of the intrahepatic bile ducts. There are two disease entities: a simple type and a periportal fibrosis type. Frequent complications with the simple type are recurrent cholangitis, liver abscess, intraductal lithiasis, abdominal pain, and fever that often lead to fatal sepsis. Development of portal hypertension and esophageal varices is usually a final feature of the periportal fibrosis type.
Caroli disease is a rare inherited disorder characterized by abnormal widening (dilatation) of the ducts that carry bile from the liver (intrahepatic bile ducts). According to the medical literature, there are two forms of Caroli disease. In most cases, the isolated or simple form is characterized by widening of the bile ducts (dilatation or ectasia). A second, more complex form is often called Caroli syndrome.
Ultrasonography is widely available, and it often is used first in the diagnosis of any process involving the liver; therefore, it is commonly performed early in the workup of patients with Caroli disease. Demonstration of the continuity of the cystic lesions with the bile ducts is paramount in diagnosis.
Caroli's disease is characterized by multifocal segmental dilatation of the intrahepatic bile ducts. It is a rare congenital condition, which appears to be autosomal recessively inherited in most cases. There are two forms of disease, one associated with congenital hepatic fibrosis and a simple form occurring alone. Recent reports suggest that the simple form may be as common as that with congenital hepatic fibrosis.
Caroli’s disease is a rare congenital condition chara-cterized 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.
Caroli's disease (CD) is a benign congenital disorder characterized by segmental cystic dilatation of the intrahepatic biliary ducts. Therapeutic strategy includes medical treatment, percutaneous, endoscopic or surgical drainage of the affected bile ducts, liver resection or transplantation. The aim of this study was to analyse the results and long-term follow-up of a consecutive series of patients who underwent surgical treatment for CD.
Caroli disease predominantly affects females. The treatment for a caroli disease depends on a number of factors including your health and the size and position of the this inherited disorder. Liver transplantation may be required. This is the best alternative when recurrent cholangitis is not present. Antibiotic treatment with broad-spectrum agents is extremely important.
Diagnosing Caroli Disease can be achieved through a non-invasive study called Magnetic Resonance Cholangiopancreatography (MRCP). During the MRCP procedure, an MRI study is completed with focus on the pancreas and biliary tractsand emphasis on the liver ducts leading to and away from the organs. It is during this non-invasive procedure that diagnosing of Caroli Disease can be confirmed.
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