Pancreaticoduodenectomy for an Intraductal Papillary Mucinous Neoplasm with Multiple Anomalies of the Pancreas

Abstract

We report an extremely rare case of an intraductal papillary mucinous neoplasm with pancreas divisum, portal annular pancreas, and bifid pancreas treated by pancreaticoduodenectomy. The patient underwent a normal pancreaticojejunostomy after pre- and intra-operative assessment of portal annular pancreas and bifid pancreas, but developed a postoperative pancreatic fistula from the stump of the transected pancreatic tissue posterior to the portal vein. We did not diagnose median arcuate ligament syndrome preoperatively, but postoperative CT showed stenosis of the celiac axis and hepatic ischemia. The patient developed a rupture of the arterial pseudoaneurysm due to the pancreatic fistula and hepatic ischemia due to median arcuate ligament syndrome, but recovered and was discharged on postoperative day 74. This case indicates that in planning a pancreatectomy for a patient with a pancreatic anomaly, the location of pancreatic transection and the appropriate method for reconstruction should be carefully considered.