Isolated congenital stenoses of the extrahepatic bile ducts. Review of the literature apropos of a new case

: The authors describe a case of congenital stenosis of the common hepatic duct, in ten-year-old girl, with jaundice for two months. Ultrasonography showed an intra and extrahepatic duct dilatation. The operative cholangiogram demonstrated severe stenosis at the end of the common hepatic duct. A Roux-en-Y anastomosis of the jejunum to this duct relieved the obstruction. Histopathological examination of the removed stricture showed non inflammatory fibrous structures. The congenital biliary strictures are exceptional. The review of the literature allowed to collect nine other cases, which symptoms began before fifteen years old. Only four were treated in childhood. The arguments for a malformative origin of the stricture are the patient's age, the seat of the stenosis and its histological structure. Several physiopathological theories allowed to explain the usually delayed beginning of first symptoms: distortion of the stenosis by gradual distension, biliary sludge, lithiasis and infection. The preoperative diagnosis of the stricture may now be made by transhepatic or endoscopic cholangiography, but before to assert its congenital feature, it must eliminate many intrinsic or extrinsic causes: post operative, traumatic, inflammatory and tumoral. The Roux-en-Y hepaticojejunostomy is the usual treatment.