A reoperation after excisional procedure was carried out in seven cases due to early or late postoperative complications. Of the 12 patients with early complications, four underwent relaparotomy due to anastomotic leakage and bleeding. Late complications were seen in nine patients with recurrent cholangitis caused by an anastomotic stricture, and three patients with intrahepatic involvement required a reoperation several years after the initial surgery. Recurrent cholangitis after biliary reconstruction mainly occurs due to an anastomotic stricture of the hepaticoenterostomy. There was no significant difference in the results between hepaticoduodenostomy and hepaticojejunostomy over a long follow-up period. A wide anastomotic stoma that permits free drainage of bile into the intestine is imperative to the prevention of cholangitis, and can be created by an incision extending along the lateral wall of both the hepatic ducts with a hepaticoenterostomy at the hilum. This procedure is obviously necessary in all patients with or without intrahepatic involvement. Carcinoma of the intrahepatic ducts and the retained distal choledochus have rarely developed in patients undergoing cyst excision followed by biliary reconstruction. Complete excision of the whole extrahepatic bile duct could prevent carcinoma arising in the distal choledochus, although it could not prevent carcinoma arising from the intrahepatic ducts. However, patients with carcinoma of the intrahepatic duct were reported to have had symptoms of biliary stricture for a long time since the cyst excision. Bile stagnation in the intrahepatic ducts is possibly responsible for the development of carcinoma. A wide anastomosis resulting in free drainage of bile appears to be essential to the prevention of carcinoma arising in the intrahepatic ducts after cyst excision.
Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall. The diagnostic criteria for pancreaticobiliary maljunction were proposed in 1987. The committee of The Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) for diagnostic criteria for pancreaticobiliary maljunction began to revise the diagnostic criteria from 2011 taking recently advanced diagnostic imaging techniques into consideration, and the final revised version was approved in the 36(th) Annual Meeting of JSPBM. For diagnosis of pancreaticobiliary maljunction, an abnormally long common channel and/or an abnormal union between the pancreatic and bile ducts must be evident on direct cholangiography, such as endoscopic retrograde cholangiopancreatography, percutaneous transpehatic cholangiography, or intraoperative cholangiography; magnetic resonance cholangiopancreatography; or three-dimensional drip infusion cholangiography computed tomography. However, in cases with a relatively short common channel, it is necessary to confirm that the effect of the papillary sphincter does not extend to the junction by direct cholangiography. Pancreaticobiliary maljunction can be diagnosed also by endoscopic ultrasonography or multi-planar reconstruction images provided by multi-detector row computed tomography. Elevated amylase levels in bile and extrahepatic bile duct dilatation strongly suggest the existence of pancreaticobiliary maljunction.
Until now, there have been no practical clinical guidelines for congenital biliary dilatation (CBD). In this review article, the Japanese Study Group on Congenital Biliary Dilatation (JSCBD) propose to establish clinical practice guidelines for CBD. Because the evidence-based literature is relatively small, we decided to create guidelines based on the consensus of experts, using the medical literature for reference. A total of 20 clinical questions (CQs) were considered by the members of the editorial committee responsible for the guidelines. The CQs included the distinct aspects of CBD: (1) Concepts and Pathology (three CQs); (2) Diagnosis (six CQs); (3) Pancreaticobiliary Complications (three CQs); Treatments and Prognosis (eight CQs). Each statements and comments for CQs were made by the guidelines committee members. CQs were finally approved after review by members of the editorial committee and the guidelines evaluation board of CBD. These guidelines were created to provide assistance in the clinical practice of CBD management; their contents focus on clinical utility, and they include general information on CBD to make this disease more widely recognized.
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