A Case of Incomplete Pancreas Divisum with Anomalous Pancreatic Duct-Biliary Duct Junction with Type IV-A Choledochal Cyst Complicated by Cystolithiasis

Abstract

A 36-year-old Vietnamese woman who suffered from migraines and anxiety presented to the ED with acute abdominal pain of 3 hrs located in the mid-epigastrium and right upper quadrant, described as sharp and severe. She had one episode of non-bloody vomiting associated with chills, was afebrile and tender to palpation in the mid-epigastrium. Lab analysis noted moderate leukocytosis (14,810 cells/mcL), mild elevations in both AST (53 U/L) and ALT (43 U/L), and normal values of alkaline phosphatase, total bilirubin, lipase and lactic acid. H. pylori serum IgG was negative. RUQ sonogram revealed a hydropic gallbladder with echogenic material and a 2.7cm shadowing stone within a 4.1cm dilated common bile duct. ERCP was performed the next day. During the procedure, the minor papilla appeared prominent with pancreatic fluid draining from the orifice. The common bile duct and to a lesser extent the common hepatic duct appeared dilated. The cystic duct also appeared mildly dilated. While injecting the distal CBD, contrast was seen filling the pancreatic duct to the body >15mm from the ampullary junction. The dorsal duct was noted to be in continuity and drained to the minor papilla. A sphincterotomy and dilation to 15mm with a CRE balloon on the sphincterotome was performed. A 10F x 7cm plastic pigtail biliary stent was placed with adequate drainage. A post-procedure MRCP revealed fusiform dilatation of the CBD with a filling defect within. The proximal portion of the left hepatic duct was also dilated measuring up to 1.4cm and corresponded to type IV A choledochal cyst by Todani classification. Two days later after receiving perioperative antibiotics she underwent resection of choledochal cyst and Roux-en-Y hepaticojejunostomy without complication. Pathology noted a gallbladder size 7.5cm in length by 5.5cm in circumference with an attached bile duct cyst 9cm in circumference by approximation. One irregular green brown calculus was found and measured 2.5 x 2.2 x 1.5cm. Microscopy of the bile duct revealed ulcerated choledochal cyst and a gallbladder with no significant histologic abnormality. A JP drain and right subcostal incision staples were removed 14 days later. Eight weeks after surgery she developed MEG pain with radiation to her back and severe sepsis. LAEs and lipase were normal and a HIDA scan was negative for obstruction of the hepatobiliary tree. She was treated with a 10-day course of antibiotics and received follow-up care with transplant surgery.Figure 1Figure 2Figure 3