Flow Diversion Surgery Research Articles

Postoperative characteristic issues of congenital dilatation of the intra- and extrahepatic bile duct

Objective: This study aimed to elucidate the postoperative particular issues of congenital dilatation of the intra- and extrahepatic bile duct (Todani type IV-A congenital choledochal cyst (CCC)) with pancreaticobiliary maljunction. Methods: 17 patients with intrahepatic stones (IHS) developed after flow-diversion surgery (excision of the extrahepatic bile duct and reconstruction) for type IV-A CCC were treated in our department between 1974 and 2015. Flow-diversion surgery was performed at our department in 6 patients and at other hospitals in 11 patients. We examined postsurgical treatment scores regarding hepatolithiasis. Results: The median time of IHS confirmation from flow-diversion surgery was 9.9 years (range: 1–20 years). Most cases presented with cholangitis. Morphologically, persistence of intrahepatic bile duct dilatation and stricture and anastomotic stricture at the hepaticoenterostomy were seen in all cases and 7 patients, respectively. Stone removal, reconstruction with choledocho-enterostomy or hepatectomy were performed for IHS. Although prognosis was good in patients with well-controlled jaundice and cholangitis, 3 patients died with liver failure due to cholestatic liver cirrhosis. Conclusion: IHS formation was considered to be largely ascribable to cholestasis caused by persistence of intrahepatic bile duct dilatation and stricture. To avoid postoperative cholestasis, adding hepatic duct plasty and creating a wide anastomosis are required when performing flow diversion surgery. Early stone removal and resolution of cholestasis to control cholangitis and jaundice is important in case IHS occurs after flow diversion surgery.

Recent advances and problems in the management of pancreaticobiliary maljunction: feedback from the guidelines committee

Clinical practice guidelines on how to deal with pancreaticobiliary maljunction (PBM) were made in Japan in 2012, representing a world first. Using a narrow definition, congenital biliary dilatation involves only Todani type I (except type Ib) and type IV-A, both of which are accompanied by PBM in almost all cases. Prospective ultrasonographic study revealed that the maximum diameter of the common bile duct increased with age. Pathophysiological conditions due to pancreatobiliary reflux occur in patients with high confluence of the pancreaticobiliary ducts, a common channel ≥ 6 mm long and occlusion of communication during contraction of the sphincter of Oddi. Since PBM can be diagnosed by magnetic resonance cholangiopancreatography, multi-planar reconstruction multi-detector row computed tomography and endoscopic ultrasonography, the current diagnostic criteria should be revised to take these diagnostic imaging modalities into consideration. According to a nationwide survey, biliary cancer occurred in 21.6% of adult patients with PBM with biliary dilatation and 42.2% of patients with PBM without biliary dilatation. In biliary cancer associated with PBM without biliary dilatation, 88.1% were gallbladder cancer. Treatment for PBM with biliary dilatation is prophylactic flow-diversion surgery, but further investigations and surveillance studies are needed to clarify the appropriate surgical strategy for PBM without biliary dilatation.

Etiology of recurrent acute pancreatitis, with special emphasis on pancreaticobiliary malformation

This study was aimed to investigate etiology and clinical profiles of recurrent acute pancreatitis (RAP), particularly from the morphology of the pancreaticobiliary duct system. Pancreaticobiliary morphology was examined in 230 of 381 patients with acute pancreatitis (AP) using endoscopic retrograde cholangiopancreatography. We analyzed factors associated with RAP including the pancreaticobiliary duct system. RAP was diagnosed in 74 patients (19%). Major etiologies of RAP were alcoholic (38%), idiopathic (26%) and pancreaticobiliary malformation (22%). Patients with alcoholic RAP were significantly younger (47.2±11.6 years) than those with gallstone RAP (67.3±16.8; p<0.05). RAP with pancreaticobiliary malformation (male-to-female ratio: 1:4.3; p<0.01) and gallstone RAP (1:1.7; p<0.05) occurred predominantly in females in comparison with alcoholic RAP (1:0.2). Recurrence rate was 80% for AP with pancreaticobiliary malformation, significantly higher than for the others (p<0.01). Pancreas divisum was suspected as the etiology of mild RAP in 7 patients. Four RAP patients with pancreas divisum underwent endoscopic minor papilla sphincterotomy and improved. Pancreaticobiliary maljunction with biliary dilatation (choledochal cyst) was suspected as the etiology of mild RAP in 3 patients. The 3 RAP patients with choledochal cyst underwent prophylactic flow diversion surgery with complete resection of the dilated common bile duct, and achieved improvement. High confluence of pancreaticobiliary ducts was suspected as the etiology of mild RAP in 6 patients. Pancreaticobiliary malformation is one of the major causes of RAP. As some of them benefit from endoscopic or surgical treatment, morphology of the pancreaticobiliary duct system should be examined where possible in RAP patients.

Forty-Year Experience With Flow-Diversion Surgery for Patients With Congenital Choledochal Cysts With Pancreaticobiliary Maljunction at a Single Institution

Forty-Year Experience With Flow-Diversion Surgery for Patients With Congenital Choledochal Cysts With Pancreaticobiliary Maljunction at a Single Institution

Forty-Year Experience With Flow-Diversion Surgery for Patients With Congenital Choledochal Cysts With Pancreaticobiliary Maljunction at a Single Institution

Congenital choledochal cyst with pancreaticobiliary maljunction (PBM) is known as a high-risk factor for various complications such as cholangitis, pancreatitis, and carcinogenesis of the biliary system by mutual refluxes of bile and pancreatic juice. Furthermore, it is not rare to suffer from postoperative complications if the wrong operative procedure is chosen. Therefore, we sought to review the relationship between operative procedure for types I and IV-A (Todani's classification) congenital choledochal cyst with PBM, and long-term treatment outcome. A retrospective review was carried out of 144 patients who underwent flow diversion surgery in our institution during the 40-year period from 1968 to 2008 and who did not have a coexisting malignant tumor at the time of surgery. Of these 144 patients, 137 underwent complete cyst excision and 7 underwent pancreas head resection as flow diversion surgery. The follow-up periods ranged from 1 to 345 months and from 1 to 271 months (average, 100.2 and 94.1) in patients with type I and type IV-A cysts, respectively. Regarding surgical treatment outcome, postoperative progress was good in 130 (90.3%) of the 144 patients. Fourteen patients required hospitalization for long-term postoperative complications such as cholangitis, pancreatitis, intrahepatic calculi, pancreatic calculus, and carcinogenesis during postoperative follow-up. Of these, 2 patients who underwent surgery for type IV-A cysts died because of secondary biliary cirrhosis with liver failure and advanced intrahepatic cholangiocarcinoma, respectively. The present study shows that flow diversion surgery for congenital choledochal cysts with PBM significantly reduces the risk of subsequent development of malignancy in the biliary tract, and it is vital to choose the appropriate operative procedure to prevent occurrence of these postoperative complications.

Pancreaticobiliary Maljunction

Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. In PBM patients, this anomaly allows regurgitation between the pancreatobiliary and biliopancreatic tract. Since hydrostatic pressure within the pancreatic duct is usually higher than that in the common bile duct, pancreatic juice frequently refluxes into the bile duct. As a result, pancreatic enzyme levels are generally very high in the bile and there is a related high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst [CCC]) and PBM without biliary dilatation (maximal diameter of the bile duct <or= 10 mm). In 42 CCC patients, bile duct and gallbladder cancer was seen in 7 and in 8, respectively. In 49 PBM patients without biliary dilatation, only gallbladder cancer was detected in 33 patients. Because of the increased cell proliferation with random K-ras mutations, the gallbladder mucosa of PBM patients should be considered premalignant. CCC treatment is prophylactic flow-diversion surgery, but only prophylactic cholecystectomy is usually performed for PBM without biliary dilatation. Pancreatobiliary reflux and premalignant changes in the gallbladder can occur in patients with a relatively long common channel (high confluence of pancreaticobiliary ducts).