La dilatation congénitale de la voie biliaire principale. A propos de 16 observations.

Congenital cystic dilatation of the common bile duct, also known as choledochal cyst, is an uncommon malformation, observed mainly in asiatic children, with a 3:1 female--male ratio. The entity is classified into 4 different types according to the anatomy of the malformation, which effects or not the intrahepatic biliary tree. The authors review theirs experience with 16 consecutive patients with choledochal cyst operated between 1971 and 1985. In 12 children (mean age: 5,8 years, 11 girls) without previous surgery, preoperative diagnosis was made with abdominal ultrasonography and, in some cases, endoscopic retrograde cholangio-wirsungography. Peroperative cholangiography demonstrated a common pancreatico-biliary channel associated with high choledochal amylase content in 6 cases. Surgical treatment consisted in cyst excision with hepatico-jejunostomy in those 12 patients. We also discuss the surgical treatment of 4 previously operated patients with postoperative cholangitis or biliary carcinoma. An anomalous pancreatico-biliary ductal junction (long common channel allowing free pancreatico-biliary reflux) is usually observed in these patients; it is considered to play a role in the etiology of congenital choledochal cysts. Appropriate surgical treatment consists in cyst excision with hepatico-jejunostomy as biliary drainage, in order to eliminate bile stasis, to remove the mechanism which allows continuous pancreatico-biliary reflux and to prevent the occurrence of bile duct carcinoma.