Late Choledochojejunostomy Dysfunction After Liver Transplantation for Primary Sclerosing Cholangitis

Abstract

A 27-year-old woman with a previous history of ulcerative colitis (UC), laparoscopic proctocolectomy with ileal J pouch–anal anastomosis, and chronic recurrent pouchitis developed end-stage liver disease secondary to primary sclerosing cholangitis (PSC) 7 years after her colectomy. Her liver disease manifested with recurrent bacterial cholangitis, pruritus, and fatigue, and she underwent an orthotopic liver transplant. The liver allograft was from a donation-after-cardiac death (DCD) donor. A Roux-en-Y choledochojejunostomy was performed for biliary reconstruction. Her postoperative course was uncomplicated until 7 years after the transplant when she presented with fever, jaundice (bilirubin 2.1 mg/dL), and elevated aspartate and alanine transaminases (AST 175 U/L, ALT 192 U/L). An ultrasonographic examination of the liver demonstrated dilated intrahepatic ducts. With a working diagnosis of biliary obstruction, an endoscopic retrograde cholangiogram via the Roux limb was planned for both diagnostic and therapeutic purposes. During the procedure, the Roux limb was entered through a slightly narrowed jejunojejunostomy site without the need for balloon dilation. The entire Roux-en-Y jejunal limb was severely ulcerated (Figure 1). The choledochojejunostomy was found to be widely patent. Because of bleeding jejunal ulcers, a therapeutic intervention was not done. To alleviate the patient's symptoms, a percutaneous transhepatic catheter was subsequently placed through the bile duct and Roux limb with the tip in the jejunum beyond the jejunojejunostomy (Figure 2) with resolution of the patient's fever, jaundice, and normalization of transaminases.Figure 2Tube cholangiogram.View Large Image Figure ViewerDownload Hi-res image Download (PPT) 1.What is the most likely cause of the ulceration and stricture of the Roux limb?a.Posttransplant lymphoproliferative diseaseb.Internal herniac.Crohn's disease (CD)d.Intestinal ischemia2.What is the appropriate management of the strictured and ulcerated Roux limb in this patient?a.Endovascular therapyb.Immediate surgical revisionc.Medical management onlyd.Medical management with surgical revision if the stricture persists3.In addition to the strictured Roux limb, what other significant finding is present in Figure 2?a.Small bowel obstructionb.Dominant stricturec.Bile leakd.Diffuse intrahepatic strictures4.What is the most likely etiology of the intrahepatic bile duct findings?a.Von Meyenburg complexb.Recurrent primary sclerosing cholangitis (PSC)c.Caroli's diseased.Ischemic cholangiopathy