Acute Pancreatitis in Children

Background

• Acute inflammation of the pancreas in childhood may be limited to a single episode, or may recur and/or progress to chronic pancreatitis.
• Causes or risk factors for acute pancreatitis (AP) in children include pancreatic and/or biliary abnormalities, medications, systemic disease, trauma, metabolic disorders, infection, and genetic mutations, and > 20% of children appear to have more than 1 such contributor.
• Persistent or minimally easing abdominal pain and epigastric tenderness is a common presentation, especially in older children and adolescents, while infants and toddlers often present with vomiting, irritability, and/or nonfocal abdominal discomfort and tenderness.
• Disease course may be mild and uncomplicated, but local or systemic complications can occur. Severe AP is associated with prolonged single or multiple organ dysfunction.

Evaluation

• Diagnose AP in children with clinical presentation meeting ≥ 2 of the following 3 criteria (Strong recommendation)
  • abdominal pain compatible with AP
  • serum amylase and/or lipase level ≥ 3 times upper limit of normal
  • imaging findings characteristic of or compatible with AP
• Diagnose acute recurrent pancreatitis in children who have had ≥ 2 distinct episodes meeting AP criteria plus either of (Strong recommendation)
  • complete resolution of pain (≥ 1 month pain-free between AP episodes)
  • complete normalization of serum amylase and lipase levels before subsequent AP episode diagnosed, as well as complete resolution of pain (regardless of duration of pain-free interval)
• Testing
  • Measure serum amylase and/or lipase levels, plus liver enzymes, triglycerides, and calcium. (Strong recommendation)
  • Imaging not required for diagnosis if blood testing confirms AP, but usually included in evaluation.
    • Transabdominal ultrasound recommended as first-line initial imaging. (Strong recommendation)
    • Perform CT or MRI if ultrasound is negative for AP and imaging diagnosis of AP is required (Strong recommendation).
    • Consider CT or MRI for identification and assessment of known or suspected complications of AP (Weak recommendation).
    • Perform MRI to identify structural or obstructive causes for acute recurrent pancreatitis (Strong recommendation).
    • Contrast-enhanced abdominal computed tomography (CT) indicated for clinical deterioration. (Strong recommendation)
    • Abdominal magnetic resonance imaging (MRI) or contrast-enhanced computed tomography (CECT) indicated if clinical presentation suggests pancreatic necrosis. (Strong recommendation)
    • Magnetic resonance cholangiopancreatography (MRCP)
      • indicated for (Strong recommendation)
        • suspected autoimmune pancreatitis.
        • initial evaluation of ARP. (Endoscopic retrograde cholangiopancreatography [ERCP] and endoscopic ultrasonography [EUS] may be alternatives depending on clinical situation.)
      • may be indicated for suspected pancreatic ductal leak or injury, or suspected biliary tract abnormalities. (Weak recommendation)
    • EUS and esophago-gastroduodenoscopy not considered standard diagnostic tools, indications for use should be determined on case-by-case basis. (Strong recommendation)
    • ERCP not indicated for diagnosis (Strong recommendation), but may be indicated as part of management in some children.
  • Additional testing in children with ARP
    • Test for genetic causes, including cystic fibrosis and PRSS1 gene mutation. (Strong recommendation)
    • Consider testing for celiac disease. (Weak recommendation)
    • Consider further causal evaluation if other etiologies suspected based on clinical presentation.

Management

• Administer IV crystalloids at 1.5-2 times maintenance for the first 24-48 hours (Weak recommendation), with initial 10-20 mL/kg bolus in children with hemodynamic compromise. (Strong recommendation)
• Start enteral nutrition (EN) as early as feasible and advance as tolerated.
  • Ideally start regular diet within 48 hours in mild AP, 72 hours in severe AP. (Strong recommendation)
  • Use parenteral nutrition (PN) in combination with EN if unable to advance EN sufficiently to meet caloric goals, or alone if unable to tolerate EN. (Strong recommendation)
• Medications may be indicated for analgesia, infection, or autoimmune AP (AIP).
  • Use opioids for analgesia if acute pain unresponsive to acetaminophen or NSAIDs. (Strong recommendation)
  • Antibiotics are indicated for suspected infected necrosis, systemic infectious complications, and cholangitis. (Strong recommendation)
  • Start oral prednisone in symptomatic children with autoimmune pancreatitis. (Strong recommendation)
• Invasive interventions may be indicated to treat complications or underlying causes of AP.
  • Endoscopic retrograde cholangiopancreatography (ERCP) is recommended for biliary pancreatitis caused by choledocholithiasis or associated with cholangitis. (Strong recommendation)
  • Cholecystectomy is indicated for gallstone pancreatitis, and should be performed during the index admission in children with mild uncomplicated acute biliary pancreatitis. (Weak recommendation)
  • Necrosectomy may be indicated to treat infected necrosis or other complications caused by necrotizing pancreatitis. Nonsurgical approaches are preferred to open procedures (Strong recommendation), and necrosectomy should be delayed as long as possible and at least 4 weeks after initial presentation. (Weak recommendation)
  • Drainage may be indicated for symptomatic pancreatic pseudocysts. Nonsurgical approaches are preferred to open drainage. (Strong recommendation)
• Total pancreatectomy with islet auto-transplantation (TPIAT) may be considered for debilitating ARP unresponsive to other interventions.