Occasionally, yellow discoloration of the skin occurs. Morbidity is common and is caused by complications of cholangitis, sepsis, choledocholithiasis , and cholangiocarcinoma. Portal hypertension may be present, resulting in other conditions including enlarged spleen , hematemesis , and melena. In a year period between and , only 10 patients were surgically treated for Caroli disease, with an average patient age of
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All rights reserved. This article has been cited by other articles in PMC. The diagnosis depends on both histology and imaging methods which can show the communication between the sacculi and the bile ducts.
Treatment consists of symptomatic treatment of cholangitis attacks by antibiotics, some endoscopic, radiological and surgical drainage procedures and surgery.
Liver transplantation seems the ultimate treatment for this disease. Prognosis is fairly good unless recurrent cholangitis and renal failure develops.
It is now clear that fibropolycystic diseases do not exist as single entities but as members of a family. The members are found in various combinations and are usually inherited. The term congenital hepatic fibrosis refers to a unique congenital liver histology characterized by bland portal fibrosis, hyperproliferation of interlobular bile ducts within the portal areas with variable shapes and sizes of bile ducts, and preservation of normal lobular architecture[ 2 ].
Many authors believe that the two conditions are actually different stages of the same disease characterized by periportal fibrosis and ductal dilatation[ 4 ]. The most acceptable theory is related to ductal plate malformation at different levels of the intrahepatic biliary tree.
Intrahepatic bile ducts develop from bipotential liver progenitor cells in contact with the mesenchyme of the portal vein which form from the ductal plates. The ductal plates are then remodeled into mature tubular ducts[ 7 ]. This process is regulated by factors influencing epithelial proliferation and apoptosis, the surrounding mesenchymal tissue, as well as the portal veins and various adhesion molecules[ 8 ].
Just as the formation of ductal plates follows the branching growth of portal vein from the hilus of the liver to its periphery, so does the remodelling process of the ductal plates beginning from the larger ducts to the smaller ducts.
The main consequences of congenital hepatic fibrosis are portal hypertension and the development of oesophageal varices. Portal hypertension may result in hematemesis or melena.
In the majority of patients, portal hypertension will not be present or will appear only later in the disease evolution[ 10 ]. Bile stagnation and hepatolithiasis explain the recurrent cholangitis which dominates the clinical course and which is the principal cause of morbidity and mortality. Chronic abdominal pain, pancreatitis resulting from biliary stones and liver abscess are other disease complications[ 10 ].
Transaminase levels may be slightly elevated. The complete blood count may reveal thrombocytopenia and leukopenia if portal hypertension and hypersplenism are present. Elevated white blood cell count or erythrocyte sedimentation rate may indicate cholangitis. BUN and creatinine values should be obtained to detect associated renal disease. The fibrovascular bundles are composed of portal vein and hepatic arteries, which can be demonstrated by Doppler ultrasonography.
MR imaging is a non-invasive technique which can confirm the diagnosis especially in the large or small cystic patterns. The use of gadolinium enhanced sequences may allow the visualization of the dot sign which seems to be very specific of the malformation of the ductal plate. MR imaging can also suggest accompanying abnormalities such as portal hypertension, cirrhosis and renal involvement[ 12 ]. The diagnosis is more difficult to establish in the case of fusiform dilatations of the biliary tracts and ERCP is the gold standard in this situation.
Intra or extra hepatic cysts can easily be detected by these methods; whereas congenital hepatic fibrosis is a histopathological diagnosis.
Maladie de Caroli