Disconnected pancreatic duct syndrome: Imaging findings and therapeutic implications in 26 surgically corrected patients

Purpose: The lack of ductal continuity between a viable pancreatic tissue and the gastrointestinal tract results in the disconnected pancreatic duct syndrome (DPDS). The purpose of our study is to describe accurately the imaging features of CT scanning and endoscopic retrograde pancreatography (ERCP) that define the DPDS. Methods: We conducted a retrospective analysis of the computed tomography (CT) and ERCP examinations in 26 consecutive patients with surgically proven disconnected pancreatic ducts treated over a 5-year period at our institution. Two abdominal radiologists concurrently defined the imaging features (presence and size of fluid collection along the course of the pancreatic duct, upstream enhancing pancreatic parenchyma, and ERCP abnormalities) via consensus for both exams. Patient demographics, etiology of pancreatitis, surgical treatment, initial CT interpretation, and the delay between symptom onset to correct diagnosis were recorded. Results: A discrete, intrapancreatic fluid collection (average size = 27 cm(2) (range, 4-74 cm(2)) along the course of the main pancreatic duct with upstream viable pancreatic parenchyma was identified by CT in 26 cases. ERCP showed ductal obstruction at the level of the intrapancreatic fluid collection in all patients with extravasation of contrast in 14 (54%). All patients were treated by operation: 15 (58%) by internal drainage into a Roux-en-Y limb of jejunum and 11 (42%) by distal pancreatic resection. No prior CT interpretation correctly identified DPDS. The average delay between symptom onset and definitive diagnosis was 9.3 months (range, 3-36 months). Conclusions: A discrete intrapancreatic fluid collection along the expected course of the main pancreatic duct with viable upstream pancreatic parenchyma suggests the diagnosis of DPDS. ERCP findings of ductal obstruction at the level of this fluid collection with or without contrast extravasation confirm this diagnosis. Treatment is surgical and requires either internal drainage or distal pancreatic resection for complete resolution.