Guideline sources The following summarized guidelines for the evaluation and management of pediatric pancreatitis are prepared by our editorial team based on guidelines from the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN 2021; 2018), the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN/NASPGHAN 2018), and the INternational Study Group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE 2018; 2017). 1 3 4 5 6 Calculator Calculator Calculat INSPPIRE diagnositc criteria for INSPPIRE diagnositc criteria for INSPPI Guidelines 1. Screening and diagnosis https://web.pathway.md/diseases/recYeYD2sT20p9Frm 1/8 6/29/23, 10:57 PM Pediatric pancreatitis Pathway Diagnostic criteria: as per NASPGHAN 2018 guidelines, diagnose pediatric acute pancreatitis according to the INSPIRE criteria. E 2. Diagnostic investigations Laboratory testing: As per NASPGHAN 2018 guidelines, obtain liver enzymes (ALT, AST, GGT, ALP, bilirubin) triglycerides, and calcium in patients with a first-time attack of acute pancreatitis. E As per INSPPIRE 2017 guidelines, obtain liver enzymes including AST, ALT, gamma- glutamyltransferase, TBIL (with fractionation if total is elevated), fasting lipids, and total serum calcium in the initial evaluation of patients with recurrent pancreatitis. B Show 2 more Cross-sectional imaging, acute pancreatitis: As per NASPGHAN 2021 guidelines, obtain transabdominal ultrasound as first-line noninvasive imaging in pediatric patients with suspected acute pancreatitis. Obtain CT or MRI if the ultrasound is negative. B Show 4 more As per NASPGHAN 2018 guidelines, consider obtaining transabdominal ultrasound as initial imaging. Reserve other imaging (CT, MRI) for more complicated cases and tailored to suspected etiology. E Cross-sectional imaging (chronic pancreatitis): obtain MRI in patients with suspected chronic pancreatitis. B Show 2 more MRCP/ERCP/EUS: As per NASPGHAN 2018 guidelines: Perform ERCP for the management of acute pancreatitis related to choledocholithiasis causing biliary pancreatitis, and for pancreatic duct pathologies, such as ductal stones or ductal leaks. E Do not obtain EUS routinely in pediatric patients with acute pancreatitis. Determine its indication on a case-by-case basis. D As per INSPPIRE 2017 guidelines, obtain MRCP in patients with acute recurrent pancreatitis. Consider obtaining ERCP or EUS as an alternative depending on the clinical scenario. A Show 2 more Genetic testing: As per INSPPIRE 2017 guidelines: Obtain a sweat chloride test (even if neonatal screening for cystic fibrosis is negative) and PRSS1 gene mutation testing for the evaluation of a genetic cause of acute recurrent or chronic pancreatitis. Consider obtaining genetic testing for cystic fibrosis if sweat testing is unavailable. A Consider obtaining mutation analysis of the genes SPINK1, CFTR, and CTRC to identify risk factors for acute recurrent or chronic pancreatitis. B Nutritional assessment: https://web.pathway.md/diseases/recYeYD2sT20p9Frm 2/8 6/29/23, 10:57 PM Pediatric pancreatitis Pathway As per ESPGHAN 2018 guidelines: Obtain routine surveillance to screen for signs of impaired growth and/or malnutrition utilizing validated measures of nutrition including weight, height, and BMI in pediatric patients with chronic pancreatitis. B Obtain specialized diabetic nutritional evaluation in pediatric patients with both chronic pancreatitis and type 3 diabetes. B Screening for celiac disease: obtain screening for celiac disease in patients with acute recurrent or chronic pancreatitis. B 3. Diagnostic procedures Upper gastrointestinal endoscopy: do not perform upper gastrointestinal endoscopy routinely in pediatric patients with acute pancreatitis. Determine its indication on a case-by-case basis. D 4. Medical management Intravenous fluids: as per NASPGHAN 2018 guidelines, administer crystalloids either with lactated Ringer or normal saline in the acute setting for initial resuscitation of pediatric patients with acute pancreatitis. Administer a bolus of 10-20 mL/kg in case of hemodynamic compromise. Provide 1.5-2 times maintenance IV fluids with monitoring of urine output over the next 24-48 hours in pediatric patients with acute pancreatitis. E Pain management: As per NASPGHAN 2018 guidelines: Administer IV morphine or other opioids for acute pancreatitis pain not responding to acetaminophen or NSAIDs. E Consult acute pain specialist services in cases of more severe pain to optimize pain management. E Pancreatic enzyme replacement therapy: as per ESPGHAN 2018 guidelines, use pancreatic enzyme replacement therapy dosing in pediatric patients with chronic pancreatitis and exocrine pancreatic insufficiency similar to recommendations established for treating exocrine pancreatic insufficiency in cystic fibrosis. B Antibiotics: As per NASPGHAN 2018 guidelines: Do not use prophylactic antibiotics empirically in patients with severe acute pancreatitis. D Administer antibiotics only in patients with acute pancreatitis with documented infected necrosis. E Corticosteroids: do not offer an empiric trial of corticosteroids in pediatric patients with acute recurrent or chronic pancreatitis in the absence of evidence suggesting autoimmune pancreatitis. D Therapies with no evidence for benefit: https://web.pathway.md/diseases/recYeYD2sT20p9Frm 3/8 6/29/23, 10:57 PM Pediatric pancreatitis Pathway Avoid using antioxidants routinely in the management of pediatric patients with acute pancreatitis. D Do not use antiproteases and probiotics in the management of pediatric patients with acute pancreatitis. D 5. Inpatient care Serial clinical and laboratory assessment: Monitor vitals at least every 4 hours during the first 48 hours of admission and during periods of aggressive hydration to monitor oxygen saturation, BP, and respiratory rate in patients admitted to an inpatient ward. Adjust the frequency based on clinical status. Obtain specialist assessment in case of abnormalities in vital signs. E Monitor BUN, creatinine, and urine output routinely during the first 48 hours as a marker of appropriate fluid management and to screen for AKI. Obtain nephrology assessment in case of abnormalities. E Serial imaging assessment (acute pancreatitis): consider obtaining an ultrasound to follow known acute pancreatitis fluid collections for resolution or progression (changes in size). C Serial imaging assessment (chronic pancreatitis): obtain MRI when clinically indicated to follow pediatric patients with acute recurrent pancreatitis and to assess for progression to chronic pancreatitis. B Show 2 more 6. Nonpharmacologic interventions Nutritional support: As per ESPGHAN 2018 guidelines, provide a regular diet in pediatric patients with chronic pancreatitis, with or without exocrine pancreatic insufficiency. B As per NASPGHAN 2018 guidelines, consider initiating early (within 48-72 hours of presentation) oral/enteral nutrition in pediatric patients with acute pancreatitis to decrease the length of stay and decrease the risk of organ dysfunction, except in the presence of direct contraindications to use the gut. E Show 2 more 7. Specific circumstances Patients with biliary pancreatitis: as per NASPGHAN 2018 guidelines, perform cholecystectomy before discharge in patients with mild uncomplicated acute biliary pancreatitis. E Patients with necrotizing pancreatitis: As per NASPGHAN 2018 guidelines: Delay interventions in patients with acute necrotic collections, even for infected necrosis, as outcomes are superior with a delayed (> 4 weeks) approach. E https://web.pathway.md/diseases/recYeYD2sT20p9Frm 4/8 6/29/23, 10:57 PM Pediatric pancreatitis Pathway Prefer nonsurgical approaches including endoscopic (EUS and ERCP-assisted) or percutaneous methods over open necrosectomy or open pseudocyst drainage when drainage or necrosectomy is necessary. E Patients with autoimmune pancreatitis (clinical presentation): Recognize that autoimmune pancreatitis is a distinct subtype of pancreatitis in pediatric patients associated with pancreatic parenchymal changes, including lymphoplasmacytic and/or neutrophilic infiltrates and/or parenchymal fibrosis, and prompt clinical response to corticosteroids is a feature of the disease. E Recognize that pediatric patients with autoimmune pancreatitis may present with acute onset of abdominal or back pain, jaundice, fatigue, and/or weight loss. E Patients with autoimmune pancreatitis (laboratory tests): recognize that pediatric autoimmune pancreatitis is associated with elevated amylase and lipase which due to a common subacute presentation may have already normalized at the time of diagnosis. E Show 2 more Patients with autoimmune pancreatitis (diagnostic imaging): Consider obtaining transabdominal ultrasound as first-line imaging in pediatric patients presenting with symptoms suggestive of pancreatitis and/or obstructive jaundice. Obtain MRI/MRCP in case of high suspicion for autoimmune pancreatitis (hypoechoic parenchyma, diffuse or focal enlargement of the pancreas, pancreatic mass lesion with/or without a dilated common bile duct in the absence of choledocholithiasis). E Suspect pediatric autoimmune pancreatitis in the presence of > 1 MRI/MRCP finding: focal, segmental, or global pancreas enlargement hypointense pancreas on T1-weighted images hypointense capsule-like rim on T2-weighted images main pancreatic duct irregularities or stricture common bile duct stricture or dilatation of the common bile duct which tapers toward an enlarged pancreatic head. E Patients with autoimmune pancreatitis (histopathology): View acute and/or chronic inflammatory cell infiltration around pancreas acini or periductular and/or presence of IgG4-positive plasma cells with or without pancreas fibrosis as suggestive of pediatric autoimmune pancreatitis. Obtain a tissue diagnosis ideally before initiating treatment. Consider diagnosing pediatric autoimmune pancreatitis based on clinical and imaging findings if barriers to obtaining a histological diagnosis cannot be overcome. E Insufficient evidence regarding the utility of major papilla biopsies for the diagnosis of pediatric autoimmune pancreatitis. I Patients with autoimmune pancreatitis (corticosteroids): recognize that some patients with pediatric autoimmune pancreatitis may have symptom resolution without any therapy. Insufficient evidence comparing complication or recurrence rate with and without treatment. Initiate oral prednisone in symptomatic patients after establishing the autoimmune pancreatitis diagnosis. E Show 2 more Patients with autoimmune pancreatitis (management of relapse): consider offering a new course of prednisone in case of relapse. Consider offering immunomodulators, such as 6- https://web.pathway.md/diseases/recYeYD2sT20p9Frm 5/8 6/29/23, 10:57 PM Pediatric pancreatitis Pathway mercaptopurine, azathioprine, mycophenolate mofetil, or infliximab (in patients with a concomitant diagnosis of IBD), as an alternative to prednisone in patients with biopsy-proven pediatric autoimmune pancreatitis, if maintenance therapy is required. E Patients with autoimmune pancreatitis (long-term surveillance): Recognize that pediatric patients with a diagnosis of autoimmune pancreatitis are at greater risk of developing other autoimmune or inflammatory diseases. Insufficient evidence about the long-term risk of complications, such as exocrine pancreatic insufficiency and diabetes. E Monitor patients with pediatric-autoimmune pancreatitis regularly by pediatric gastroenterologists, and when reaching adulthood, by adult gastroenterologists. E Patients with congenital pancreatic anomalies: recognize that pancreas divisum and annular pancreas are risk factors for acute recurrent pancreatitis but alone is not sufficient to prevent evaluation for concomitant causes of acute recurrent or chronic pancreatitis. B 8. Follow-up and surveillance Surveillance for complications: As per ESPGHAN 2018 guidelines, monitor for pancreatic exocrine insufficiency every 6-12 months with fecal elastase or 72-hour fecal fat collection in pediatric patients with chronic pancreatitis. B Show 5 more As per NASPGHAN 2018 guidelines, obtain close follow-up to identify early or late complications or recurrence in pediatric patients with acute pancreatitis. E As per INSPPIRE 2017 guidelines: Monitor for fat-soluble vitamin deficiency, B pancreatic exocrine insufficiency, and pancreatic endocrine insufficiency at least annually in pediatric patients with acute recurrent peritonitis. B Monitor for fat-soluble vitamin deficiency and pancreatic exocrine insufficiency at least annually in pediatric patients with chronic peritonitis. B Clinical findings Patient demographics Symptoms Children Abdominal discomfort Abdominal pain Past medical history Back pain Diarrhea Autoimmune pancreatitis Epigastric pain Celiac disease Fatigue Congenital biliary anomalies Fever Congenital pancreatic anomalies Flatulence Irritability Cystic fibrosis Steatorrhea Diabetes mellitus https://web.pathway.md/diseases/recYeYD2sT20p9Frm 6/8 6/29/23, 10:57 PM Pediatric pancreatitis Pathway Gallstones Vomiting HUS Weight loss Henoch-sch nlein purpura Past events IBD Kawasaki disease Blunt abdominal trauma SLE Abdominal exam Sepsis Abdominal distension Vital signs Abdominal tenderness Hypotension Lab findings Tachycardia Diabetic ketoacidosis Integument exam blood glucose Jaundice serum ALT serum AST Genetic testing serum TBIL Cftr gene mutation serum amylase Prss1 gene mutation serum calcium Spink1 gene mutation serum gamma- glutamyltransferase serum lipase serum triglycerides References 1. Maisam Abu-El-Haija, Soma Kumar, Jose Antonio Quiros et al. Management of Acute Pancreatitis in the Pediatric Population: A Clinical Report From the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition Pancreas Committee. J Pediatr Gastroenterol Nutr. 2018 Jan;66 1 159 176. Open 2. P rniczky A, Abu-El-Haija M, Husain S et al. EPC/HPSG evidence-based guidelines for the management of pediatric pancreatitis. Pancreatology. 2018 Mar;18 2 146 160. Open 3. Cheryl E Gariepy, Melvin B Heyman, Mark E Lowe et al. Causal Evaluation of Acute Recurrent and Chronic Pancreatitis in Children: Consensus From the INSPPIRE Group. J Pediatr Gastroenterol Nutr. 2017 Jan;64 1 95 103. Open 4. Andrew T Trout, Sudha A Anupindi, A Jay Freeman et al. North American Society for Pediatric Gastroenterology, Hepatology and Nutrition and the Society for Pediatric Radiology Joint Position Paper on Noninvasive Imaging of Pediatric Pancreatitis: Literature Summary and Recommendations. J Pediatr Gastroenterol Nutr. 2021 Jan 1;72 1 151 167. Open 5. Maisam Abu-El-Haija, Aliye Uc, Steven L Werlin et al. Nutritional Considerations in Pediatric Pancreatitis: A Position Paper from the NASPGHAN Pancreas Committee and ESPGHAN Cystic Fibrosis/Pancreas Working Group. J Pediatr Gastroenterol Nutr. 2018 Jul;67 1 131 143. Open 6. Isabelle Scheers, Joseph J Palermo, Steven Freedman et al. Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus From INSPPIRE. J Pediatr Gastroenterol Nutr. 2018 Aug;67 2 232 236. Open https://web.pathway.md/diseases/recYeYD2sT20p9Frm 7/8 6/29/23, 10:57 PM Pediatric pancreatitis Pathway 7. P rniczky A, Abu-El-Haija M, Husain S et al. EPC/HPSG evidence-based guidelines for the management of pediatric pancreatitis. Pancreatology. 2018 Mar;18 2 146 160. Open 8. Veronique D Morinville, Sohail Z Husain, Harrison Bai et al. Definitions of pediatric pancreatitis and survey of present clinical practices. 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