[Single-lobe Caroli's disease. Anatomoclinical aspects. Diagnostic and therapeutic procedure. Apropos of 3 personal cases and 101 cases in the literature]

Abstract

On the basis of 3 personal cases of single-lobe Caroli's disease and of 101 cases in the literature, the authors have observed that biliary-type pain was the most constant presenting symptom (85%), in association with fever (72%), while angiocholitis was observed in only 44% of all cases. The preoperative diagnosis of the disease and of the hepatic and extrahepatic lesions is aided by the noninvasive techniques of morphological exploration such as ultrasound and computed tomography, which must be proposed in first intention as they clearly demonstrate the cystic nature of the intrahepatic lesions, the associated biliary lesions [choledochal cyst (30%), lithiasis (37%)]. However, the cystobiliary communication and the definition of the type of biliary dilatation can be demonstrated only by transhepatic percutaneous cholangiography, endoscopic retrograde cholangiography, the injection of a contrast medium with biliary elimination, and quite often by intraoperative cholangiography only. The 4 types of single-lobe Caroli's disease distinguished by the authors, ie: type I: racemose biliary dilatation, type II: digitiform biliary dilatation, type III: large cystic biliary dilatation, type IV: choledochal cyst associated to the intrahepatic biliary disease, require a particular surgical treatment, which must also take account of the operative risks and of all the associated lesions (lithiasis, choledochal cyst, congenital hepatic fibrosis (5 cases), neoplastic degeneration (8 cases)]. External biliary bypass no longer has any use for treatment as it constantly fails. First-intention hepatic resection is the ideal treatment is all anatomical forms of single-lobe Caroli's disease as it treats all the hepatic lesions with no mortality. It is therefore indicated whenever the general conditions do not produce an excessive risk, when the controlateral liver is normal and when resection will not injure the bile duct of the remaining liver. If an associated choledochal cyst is present, it must be resected at the same time. Hepatic resection also is the best second surgery to propose when the other methods have failed. Hepaticojejunal anastomosis is the therapeutic solution every time hepatic resection cannot be performed.