What is Disconnected Pancreatic Duct Syndrome (DPDS)?

Disconnected Pancreatic Duct Syndrome (DPDS)

Disconnected Pancreatic Duct Syndrome (DPDS) is a serious complication of acute necrotizing pancreatitis characterized by complete disruption of the main pancreatic duct with viable upstream pancreatic tissue that continues to secrete pancreatic juice but is no longer in continuity with the gastrointestinal tract. 1

Definition and Pathophysiology

DPDS occurs when necrosis of the pancreatic parenchyma leads to complete disruption of the main pancreatic duct, resulting in:

• Disconnection between viable upstream pancreatic tissue and the duodenum
• Continued secretion of pancreatic juice from the disconnected segment
• Inability of pancreatic secretions to reach the gastrointestinal tract 2

This condition typically develops as a consequence of:

• Acute necrotizing pancreatitis (most common cause)
• Pancreatic trauma
• Chronic pancreatitis (less frequently) 3

Clinical Presentation

DPDS presents in three distinct forms:

1. Concurrent DPDS: Diagnosed simultaneously with acute necrotizing pancreatitis
2. Delayed DPDS: Presents months after the initial episode as a pseudocyst
3. Chronic Pancreatitis DPDS: Develops as a complication of chronic pancreatitis 3

Common clinical manifestations include:

• Recurrent pancreatic fluid collections
• Persistent external pancreatic fistulae
• Chronic abdominal pain
• Recurrent episodes of pancreatitis 2

Diagnosis

The diagnosis of DPDS requires a high index of suspicion and appropriate imaging studies:

• ERCP: Historically considered the gold standard, showing complete cutoff of the pancreatic duct
• Secretin-enhanced MRCP: Recommended as an appropriate diagnostic method in current guidelines
• Contrast-enhanced CT: Shows viable enhancing distal pancreas with disconnection
• EUS: Increasingly used for diagnosis and subsequent management 4, 5

Diagnostic criteria include:

• Complete disruption of the main pancreatic duct
• Viable pancreatic tissue upstream from the disruption
• Extravasation of contrast during ERCP 1, 2

Management Approaches

Management of DPDS is complex and often requires a multidisciplinary approach:

Endoscopic Management

• Transmural drainage: EUS-guided drainage of pancreatic fluid collections
• Transpapillary stenting: Attempted when partial duct continuity exists
• Combined approaches: Using both transmural and transpapillary techniques 4

Surgical Management

Based on presentation type:

1. Concurrent DPDS: Necrosectomy with distal pancreatectomy within 60 days of onset
2. Delayed DPDS: Distal pancreatectomy (typically performed months after diagnosis)
3. Chronic Pancreatitis DPDS: Lateral pancreaticojejunostomy 3

Surgical options include:

• Internal drainage: Roux-en-Y drainage procedures
• Resection: Distal pancreatectomy when appropriate
• Combined approaches: Based on individual anatomy and extent of disease 6

Step-up Approach for Infected Necrosis

For infected pancreatic necrosis with DPDS:

1. Percutaneous catheter drainage as first-line treatment
2. Endoscopic drainage if percutaneous approach fails
3. Minimally invasive surgical necrosectomy
4. Open surgical necrosectomy as last resort 1

Complications and Outcomes

• Mortality: Approximately 2% with appropriate management
• Morbidity: Up to 46% overall
• Pancreatic fistula: Occurs in 7-36% of cases depending on presentation and management
• Recurrence: Approximately 11% of patients require repeat pancreatic intervention 6, 3

Important Considerations

• Timing of intervention is critical, with delayed intervention (>4 weeks after onset of pancreatitis) resulting in lower mortality 1
• The presence of sinistral portal hypertension due to splenic vein thrombosis complicates surgical management
• Parenchymal volume of disconnected pancreas influences treatment decisions
• Endoscopic approaches are increasingly preferred as first-line treatment over traditional surgical approaches 4, 2

Early recognition and appropriate classification of DPDS are essential for optimal management and improved outcomes. The treatment approach should be tailored based on the specific presentation, anatomical considerations, and available expertise.