[Cystic dilatation of main biliary tract]

The choledochal cyst (particularly with congenital origin) is a clinical form from a complex syndrome of ecstasies of the intra-, extra-, or both situations of biliary tree. The disease has until now an unsure etiopathogeny. Very frequent to child, teen-ager and young adult the incidence decreases through the third age. During the period 1960-1998, 13 patients were operated on (annually rate 0.3). The extreme ages of our patients were 5 and 72 years; being operated six children and 7 adults (two of them being 63 and 72 years old). The clinical presentation was nonspecific. The jaundice (with or without cholangitis) (n: 7), right upper quadrant pain (n: 11), a palpable abdominal mass (n: 5) were registrated but the classic triad (Sénéque--Thailhefer) was in out series the exception rather than the rule. Concomitantly there were discovered different complications of the disease: hepatic biliary cirrhosis (n: 3--two macroscopic diagnosed and one microscopic confirmed), portal hypertension (n: 8), biliary lithiasis (including the intracystic stones) (n: 6) and cholangio-carcinoma (n: 1). The clinic and biologic preoperative diagnosis is up to date difficult and not sure like in our experience too. In our series, especially in the last 20 years, the accurate imaging diagnosis was helpful (the ultrasonography, CT, hepatic scintigrams). ERCP was not available for us until now. In concordance with Todani--Watanabe classification our patients were included in the following types: I-a (n: 6), I-b (n: 1), I-c (n: 2), II (n: 2), IV-a (n: 2). All patients were operated on: cysts-digestive derivations or partial excision of the cyst with billio-digestive reconstruction were the choice procedures at a beginning of our experience or for the very fragile patients (n: 5). The total excision of the cyst with choledochal--jejunostomy (Roux-en-Y) is the preferred method now (n: 8) when it is possible. The microscopic pathology of the disposable specimens (cystic wall) showed:--chronic intra- and perimural inflammation (n: 7);--severe displasia and metaplasia (n: 2); cholangiocarcinoma islands (n: 1); additional lesions: hepatic chirrosis (n: 1); hepatic fibrosis (n: 3).

The postoperative morbidity--three patients--one of them died with a peritonitis. Long-term follow-up, effective for only 10 patients, reveled recurrent cholangitis in two cases treated by the reoperation of the former anastomosis; the other 8 patients had a normal evolution.

The choledochal cyst is an exclusive indication for surgery as soon as it was discovered.