What is the initial approach to managing a patient with pancreatic divisum?

Initial Management of Pancreas Divisum

For patients with symptomatic pancreas divisum, endoscopic ultrasound (EUS) is the preferred initial diagnostic test, followed by endoscopic retrograde cholangiopancreatography (ERCP) with minor papilla endotherapy for those with recurrent acute pancreatitis and objective signs of outflow obstruction such as dilated dorsal pancreatic duct. 1

Understanding Pancreas Divisum

Pancreas divisum is the most common congenital pancreatic anomaly, occurring in approximately 6-10% of the general population. It results from failure of fusion between the dorsal and ventral pancreatic ductal systems during embryogenesis, leading to dominant pancreatic duct drainage through the minor papilla.

Clinical Presentation

• Most individuals with pancreas divisum are asymptomatic
• Only about 5% develop symptoms, which may include:
  • Recurrent acute pancreatitis (RAP)
  • Chronic pancreatitis
  • Chronic abdominal pain

Diagnostic Approach

1. Initial Evaluation:

   • Rule out common causes of pancreatitis (gallstones, alcohol, hypertriglyceridemia, medications)
   • Obtain serum amylase/lipase, liver function tests, triglycerides, and calcium levels 1

2. First-line Imaging:

   • EUS is the preferred initial diagnostic test for unexplained acute and recurrent pancreatitis 1
   • Should be performed 2-6 weeks after resolution of acute pancreatitis to avoid interference from inflammatory changes

3. Complementary Imaging:

   • MRI with MRCP (magnetic resonance cholangiopancreatography) is particularly helpful for identifying pancreatic ductal anomalies including pancreas divisum 1
   • Secretin-enhanced MRCP may improve diagnostic yield but has limited availability

Management Algorithm

For Asymptomatic Patients:

• No intervention required

For Symptomatic Patients with Recurrent Acute Pancreatitis:

1. Initial Management:

   • Conservative treatment of acute episodes (fluid resuscitation, pain control, nutritional support)

2. Endoscopic Intervention (for patients with recurrent episodes):

   • ERCP with minor papilla endotherapy should be considered, particularly for those with:
     • Objective signs of outflow obstruction
     • Dilated dorsal pancreatic duct
     • Santorinicele (cystic dilatation of the terminal portion of the dorsal duct) 1

3. Endoscopic Techniques:

   • Minor papilla sphincterotomy
   • Balloon dilation
   • Temporary stent placement
   • Note: Stent placement alone is not recommended as definitive therapy due to need for frequent exchanges and risk of stent-related ductal injury 1

4. Surgical Options (when endoscopic therapy fails):

   • For soft pancreas without fibrosis: Reinsertion of the papilla (sphincteroplasty)
   • For fibrotic/inflammatory pancreas: Duodenum-preserving pancreatic head resection
   • For extensive disease: Pylorus-preserving Whipple procedure 2

Efficacy and Outcomes

• Endoscopic therapy has shown success in reducing or eliminating episodes of acute pancreatitis in observational studies, but high-quality randomized controlled trials are limited 1
• One small randomized trial (19 patients) showed minor papilla stenting reduced future episodes of acute pancreatitis compared to controls (10% vs 67%) 1
• Surgical treatment has demonstrated significant pain reduction in patients with symptomatic pancreas divisum when performed according to individualized approach based on pancreatic morphology 2

Important Considerations

• Potential Risks:

  • Post-ERCP pancreatitis (10-15% risk)
  • Stent migration/occlusion
  • Need for repeated procedures

• Long-term Follow-up:

  • Patients may require restenting for recurrence of pain (approximately 39% in one study) 3
  • Some patients may eventually need surgical intervention despite initial endoscopic success

• Ongoing Research:

  • An international, multicenter, randomized sham-controlled trial evaluating minor papilla sphincterotomy in patients with pancreas divisum and recurrent acute pancreatitis is currently underway 1

Remember that the decision to proceed with endoscopic therapy should be made after careful consideration of potential adverse events and a thorough discussion with the patient about the uncertain benefits and risks.