Disconnected Pancreatic Duct Syndrome

Disconnected pancreatic duct syndrome (DPDS) is complete transection of the main pancreatic duct with a viable upstream pancreatic remnant (tail), whose secretions can no longer reach the duodenum.

• Complicates 30 to 50% of patients with necrotising pancreatitis, and occurs after trauma or pancreatic surgery.
• Pathophysiology: central gland necrosis disconnects the tail, which continues to secrete, causing persistent collections, fistula, ascites or pseudocyst.
• Recurrent collections, pancreatic atrophy of the disconnected segment, left sided portal hypertension.
• Complications: recurrent fluid collections and infection, external pancreatic fistula, pancreatic ascites, recurrent pancreatitis.

• Recurrent or persistent peripancreatic collections after necrotising pancreatitis, persistent pain, weight loss.
• Recurrent infection and fever, external drain output of pancreatic fluid.
• Left sided portal hypertension with splenic vein thrombosis.
• Exocrine and endocrine insufficiency.

• Secretin enhanced MRCP is the key test: discontinuity of the main pancreatic duct with a viable tail that continues to secrete fluid (leakage on imaging after secretin).
• Contrast CT: necrosis of the pancreatic neck or body with a viable enhancing tail, collection in the lesser sac.
• ERCP: complete duct cut-off at the site of necrosis; cannot pass a guidewire beyond.
• Endoscopic ultrasound for characterising collections.
• Biochemistry of drain fluid: high amylase.
• Assess exocrine function (faecal elastase) and diabetes.

• Endoscopic transmural drainage is the cornerstone: EUS guided cystogastrostomy with LAMS (15 to 20 mm) or multiple plastic double pigtail stents, with necrosectomy if needed.
• Long term indwelling plastic double pigtail stents (rather than removing LAMS) reduce recurrence of collections (from over 40% to under 10%): replace LAMS with plastic stents at 3 to 4 weeks, leave in place indefinitely, with periodic imaging and exchange only if symptomatic.
• Transpapillary stenting is generally ineffective for complete disconnection.
• Percutaneous drainage in unfit patients or inaccessible collections (risk of external fistula); convert to internal drainage (percutaneous endoscopic).
• Surgery for failure or recurrent disease: distal pancreatectomy with splenectomy (most definitive for a tail remnant, recurrence nearly eliminated), or Roux-en-Y cystojejunostomy or pancreaticojejunostomy to the disconnected tail (preserves endocrine function).
• Antibiotics for infection (piperacillin tazobactam 4.5 g every 6 hours); nutritional support (enteral feeding).
• Pancreatic enzyme replacement therapy and glycaemic management.
• Follow up imaging at 3 to 6 months and yearly.