What is the best course of action for a patient in their 50s with a history of acute pancreatitis secondary to a large intraductal papillary mucinous neoplasm (IPMN), presenting with worsening epigastric pain, fevers, nausea, cholestatic injury, elevated lipase, and hyponatremia?

Urgent ERCP for Biliary Decompression and Multidisciplinary Evaluation

This patient requires urgent ERCP within 24 hours for biliary decompression given the cholestatic injury pattern (elevated alkaline phosphatase 232, bilirubin 1.4, R-factor 1.2) with fever, which raises concern for cholangitis or biliary obstruction from the large IPMN compressing or involving the biliary tree. 1

Immediate Management Priorities

Urgent Diagnostic Workup

• Obtain contrast-enhanced CT (CECT) abdomen immediately to assess for:

  • Pancreatic necrosis (though lipase of 232 suggests mild pancreatitis compared to prior 780) 1
  • Biliary ductal dilatation or obstruction 1
  • IPMN characteristics: main duct involvement, mural nodules, or interval growth 1
  • Complications such as infected necrosis or fluid collections 1

• Check procalcitonin level as it is the most sensitive laboratory test for detecting pancreatic infection, with low values being strong negative predictors of infected necrosis 1

• Monitor for organ failure criteria including persistent hypotension, respiratory failure (PaO2/FiO2 <300), or renal dysfunction (creatinine >1.9 mg/dL) for >48 hours, which would classify this as severe acute pancreatitis requiring ICU transfer 1

ERCP Timing and Indications

The decision to perform urgent ERCP is straightforward in this case given the cholestatic pattern with fever suggesting possible cholangitis or biliary obstruction from the IPMN 1. The guidelines state that for patients with presumed malignant biliary obstruction (which large IPMNs can cause), biliary decompression with ERCP should be performed urgently 1.

Key considerations for ERCP:

• The lipase elevation (232) is modest compared to the prior admission (780), suggesting this may be more biliary obstruction than acute pancreatitis recurrence 1
• Fever with cholestatic injury raises concern for ascending cholangitis, which requires urgent biliary drainage 1, 2
• ERCP can simultaneously assess for main duct involvement and provide therapeutic decompression 1

IPMN-Specific Considerations

Risk Stratification

This patient's presentation is concerning for high-risk IPMN features:

• Symptomatic presentation with recurrent pancreatitis is associated with main duct involvement (OR 1.87), high-grade dysplasia (OR 1.82), and malignancy (OR 1.97) 3
• Acute pancreatitis in IPMN patients occurs in 12-67% of cases and is more common with main duct or combined-type IPMNs (14% vs 5% for branch duct) 4, 5
• Intestinal subtype IPMNs (which produce highly viscous MUC2-containing mucin) are strongly associated with acute pancreatitis (OR 4.84) and have higher malignant potential 3

Sendai Guidelines Application

Per the International Association of Pancreatology Sendai guidelines, surgical resection is recommended for:

• All main duct IPMNs 1
• Branch duct IPMNs >3 cm 1
• IPMNs with mural nodules 1
• Symptomatic IPMNs (including those causing pancreatitis) 1, 4

This patient meets criteria for surgical evaluation given the symptomatic presentation with recurrent pancreatitis and the "large" IPMN designation 1.

Medical Management During Acute Phase

Pancreatitis Management

• Defer definitive IPMN intervention (surgical resection or pancreatic duct drainage) until the acute inflammatory phase resolves 1
• Medical management should be optimized with goal of avoiding procedures unless there is deteriorating clinical status or signs of impending sepsis 1
• Monitor for persistent organ failure (>48 hours), which would upgrade severity classification and require ICU care 1

Supportive Care

• Aggressive IV fluid resuscitation targeting urine output >0.5 mL/kg/hr 1
• Correct hyponatremia cautiously (likely dilutional from fluid shifts) 2
• NPO status initially, advance diet as tolerated when pain improves 1
• Antiemetics for nausea: ondansetron 8 mg sublingual every 4-6 hours (obtain baseline ECG first for QTc) 2

Antibiotic Considerations

If cholangitis is confirmed (fever + cholestatic pattern + biliary obstruction), start empiric antibiotics immediately:

• Broad-spectrum coverage pending ERCP and cultures 1
• Do NOT use prophylactic antibiotics for pancreatitis alone unless infected necrosis is documented 1

Multidisciplinary Planning

Tumor Board Presentation

This case requires urgent multidisciplinary discussion including gastroenterology, hepatobiliary surgery, and medical oncology 1. Key decision points:

1. Timing of surgical resection after acute episode resolves (typically 4-6 weeks) 1
2. Extent of resection based on IPMN location and main duct involvement 1
3. Need for tissue diagnosis via EUS-FNA if not already obtained, though prior FNA diagnosed IPMN 1

Post-ERCP Surveillance

If ERCP successfully decompresses the biliary tree and surgery is deferred:

• Repeat imaging in 3-6 months to assess IPMN stability 1
• Lifelong surveillance is required even after partial pancreatectomy due to risk of metachronous lesions in remnant pancreas 1
• Screen for extrapancreatic malignancies (colon, stomach) as IPMN patients have increased risk 1

Critical Pitfalls to Avoid

• Do not attribute pancreatic cysts to pseudocysts in patients with known IPMN—this delays appropriate management 4
• Do not delay ERCP when cholestatic injury with fever is present, as this may represent cholangitis requiring urgent drainage 1, 2
• Do not perform complex interventional procedures (EUS-guided ERCP, tumor resection) during acute inflammatory phase due to increased complication risk 1
• Do not assume mild lipase elevation excludes significant pathology—the cholestatic pattern and fever are more concerning than the lipase level alone 1, 2
• Recurrent pancreatitis in IPMN does not correlate with malignancy risk but is an independent indication for resection per Sendai guidelines 4, 5, 3