Outcomes of Endoscopic Therapy for Anomalous Pancreatobiliary Duct Junction (APBDJ) Ramanujan Samavedy, Evan L. Fogel, Lee Mchenry, Stuart Sherman, James L. Watkins, Suzette E. Schmidt, Glen A. Lehman Background: APBDJ is a rare finding in non-Asian population. We reported our experience with endotherapy for APBDJ (Gastrointest Endos. 1999; 50:623-627). This is an update. Methods: Review of our ERCP database from 1988 to 2005 yielded 45 APBDJ patients from approximately 17,000 patients entered. These patients were evaluated for therapeutic response by telephone calls, review of database and charts. Three patients with malignancies were excluded from analysis. Mean follow up was 36 months (2 months 10 yr). Rationale for therapy: APBDJ is associated with reflux of bile into the pancreatic ductal system. Common channel endoscopic sphincterotomy is thought to divert bile away from the pancreas. Results: There were 12 males and 30 females. Indications for ERCP were chronic abdominal pain with or without abnormal serum liver chemistries or pancreatitis. The ERCP findings: 26 patients had chronic pancreatitis, 15 had choledochal cysts, 17 with biliary or pancreatic strictures and 17 patients with biliary stones. Twenty-seven patients without choledochal cyst underwent primary endoscopic therapy: 22 had common channel sphincterotomy, 15 had stricture dilation and or stent placement. At latest follow up, 16 (59%) patients remain symptom free after endotherapy alone, 6 of these patients now have more than 6 years follow up. Six (22%) additional patients are improved symptomatically with endotherapy alone, requiring only occasional home analgesics. Three of these have more than 6 years follow up. Five patients did not respond to initial endotherapy and had additional interventions including liver transplantation, serial stent placement and open sphincterotomy. Endoscopic complications were mild pancreatitis in 4 and mild bleeding in 1. Of the fifteen patients with choledochal cyst, 14 underwent cyst resection initially and all are asymptomatic. One awaits cyst surgery. Summary: APBDJ is associated with choledochal cysts, pancreatic and biliary strictures, biliary stones and chronic pancreatitis. In this series of 42 APBDJ, 27 patients underwent endoscopic therapy and 22 (81%) appear to have improvement of pain, pancreatitis or number of hospitalization, over a mean follow up of 3 years. Nine patients had sustained improvement for at least 6 years. Choledochal cyst was present in 15 patients and was managed surgically. Conclusions: Endoscopic therapy is clinically helpful in majority of APBDJ patient without choledochal cysts presenting with chronic abdominal pain or pancreatitis.