Chronic Pancreatitis in Children

Background

• Chronic pancreatitis (CP) is a progressive inflammatory process that leads to destruction of the pancreatic parenchyma, resulting in irreversible changes in pancreatic structure and causing pancreatic dysfunction.
• Causes or risk factors for CP in children include genetic variants (common), acute recurrent pancreatitis (ARP), pancreatic and/or biliary obstruction, systemic disease, metabolic disorders, and medications; > 20% of children appear to have > 1 such contributor.
• CP may present with a history of abdominal pain consistent with pancreatitis and/or symptoms or signs of pancreatic dysfunction, and most CP is preceded by ARP.
• Pain and complications can cause substantial disease burden, often involving recurrent emergency department visits and hospitalizations, missed school days, and invasive interventions.

Evaluation

• Diagnose CP in children meeting the following criteria (Strong recommendation):
  • Any of:
    • periods of abdominal pain consistent with pancreatitis or amylase or lipase ≥ 3 times upper limit of normal
    • exocrine pancreatic insufficiency
    • endocrine pancreatic insufficiency
  • Plus irreversible structural changes in the pancreas such as diffuse or focal destruction, sclerosis, or pancreatic duct abnormalities/obstruction.
• Perform pancreatic imaging to identify characteristic structural changes, help identify etiology, and assess for complications. (Histopathologic diagnosis is rare, but possible if surgical or biopsy specimen is available.)
  • Consider magnetic resonance imaging when CP is suspected as part of the initial diagnosis (Weak recommendation).
  • Obtain a transabdominal ultrasound when imaging is required to evaluate a suspected or known episode of acute pancreatitis in a child with CP (Strong recommendation).
  • Computed tomography (CT) can detect parenchymal changes, but involves exposure to ionizing radiation and has poor sensitivity for ductal abnormalities.
  • Endoscopic ultrasonography (EUS) can detect parenchymal disease and ductal abnormalities.
• Diagnosis of exocrine pancreatic insufficiency is typically based on fecal elastase or fat measurements, less commonly on invasive pancreatic function testing.
• Diagnosis of endocrine pancreatic insufficiency is typically based on fasting blood glucose, HbA1c, or glucose tolerance testing.
• Additional causal evaluations
  • Perform blood testing, including liver enzymes, bilirubin, fasting lipids, and total serum calcium (Strong recommendation).
  • Test for genetic causes, including cystic fibrosis and PRSS1 gene variant (Strong recommendation).
  • Consider testing for celiac disease (Weak recommendation).
  • Consider testing for other etiologies suspected based on clinical presentation.

Management

• Management depends on symptoms and/or underlying cause, and should include close monitoring for and treatment of common complications such as exocrine pancreatic insufficiency (EPI), type 3c diabetes mellitus (T3cDM), and vitamin deficiency.
• Treat attacks of acute pancreatitis the same as in children presenting with initial acute pancreatitis episode. (Strong recommendation)
• Dietary management should include a regular diet (standard proportions of protein, fat, and carbohydrate) in children with and without EPI, and a specialized diabetic nutritional evaluation in children with T3cDM (Strong recommendation).
• Nonnarcotic analgesics should be first-line treatment for pain, with narcotics reserved for pain uncontrolled by nonnarcotic medication (Strong recommendation).
• Other medications that may be indicated to treat complications or underlying cause include:
  • pancreatic enzyme replacement therapy (PERT) for EPI
  • insulin for diabetes mellitus
  • corticosteroids for autoimmune pancreatitis
• Therapeutic interventions to consider may include:
  • endoscopic retrograde cholangiopancreatography (ERCP) to treat duct obstruction and other complications
  • extracorporeal shock wave lithotripsy (ESWL) to treat pancreatic stones (ESWL may be followed by endoscopic extraction of fragments)
  • surgery to treat symptoms unresponsive to medical or endoscopic interventions; options include:
    • conventional drainage and resection procedures (for example, pancreatojejunostomy [Puestow procedure])
    • total pancreatectomy with islet auto-transplantation (TPIAT) for debilitating chronic pancreatitis unresponsive to other interventions