Abstract

Cystic-cylindrical dilatation of the intrahepatic and extrahepatic bile ducts occurs rarely in childhood. Aetiologically, congenital pancreaticobiliary junction anomalies play a decisive part. Accurate preoperative diagnosis is by no means an easy matter. The best possible diagnostic approach to clarify the pathologico-anatomic conditions consists in sonography coupled with on-target partial intraoperative cholangiography presenting the preduodenal section of choledochus and pancreatic duct. Between 1979 and 1987 surgery was performed at the Paediatric Surgical Department of the Municipal Paediatric Hospital of Cologne on 18 patients suffering from intrahepatic and extrahepatic cysticocylindrical dilatation of the bile ducts due to confirmed ectopic pancreaticobiliary junction anomaly. This study does not include all other types of choledochus cysts or biliary duct dilatations without proven pancreaticobiliary junction anomaly. The treatment of choice is the resection of the dilated extrahepatic biliary ducts followed by hepatico-jejunostomy using the Roux-en-Y technique. To avoid cholangitis due to reflux of intestinal contents via the shunted jejunum loop, we are constructing a two-stage anti-refluxive muscular mucosa valve in the shunted loop; this practice has been followed by us since 1983. Permanent postoperative freedom from cholangitis in 10 children after construction of the valve prompts us to recommend this procedure also in other types of bile duct surgery, especially in the treatment of atresias.

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