Guidelines Summary
European Pancreatic Club and Hungarian Pancreatic Study Group
Acute pancreatitis (AP): The first acute episode of pediatric pancreatitis occurs before the age of 18 years. The diagnosis of AP is made by meeting at least two of the following three criteria: (1) abdominal pain; (2) serum lipase or serum amylase level at least 3 times greater than the upper limit of normal; (3) characteristic findings of AP with imaging methods.
Diffuse, epigastric, persistent, or minimally easing abdominal pain suggests AP in childhood.
In children, especially in infants and in toddlers, vomiting, irritability, abdominal distention may suggest AP.
Measurement of serum amylase and/or lipase among the routine laboratory tests is recommended in the presence of abdominal pain
Etiological factors that should be considered after the diagnosis is reached are the following: biliary and pancreatic abnormalities, medication-associated, presence of underlying systemic disease, trauma, genetic predisposition, infection, metabolic disorders, and autoimmune pancreatitis.
In patients with a second episode of idiopathic AP or a first episode of idiopathic AP and a family history of AP or chronic pancreatitis (CP), full sequence analysis of the PRSS1, CPA1, SPINK1, CTRC, and CFTR gene exons and exon-intron boundaries and testing for the CEL gene pathogenic hybrid allele is recommended in order to explore the etiologic background.
Transabdominal ultrasonography is recommended as a first-choice imaging technique in pediatric AP.
Contrast-enhanced abdominal computed tomography (CT) is recommended in clinical deterioration in children as per adult guidelines.
If the clinical presentation of the child suggests pancreatic necrosis, contrast-enhanced abdominal CT or magnetic resonance imaging (MRI) is recommended, as per adult guidelines. The optimal timing of the scan is at least 72–96 hr after presentation with pancreatitis.
Endoscopic retrograde cholangiopancreatography (ERCP) is not recommended for diagnostic purposes.
Administration of dextrose containing crystalloids is recommended as the initial choice for replacement fluid therapy in AP.
Due to lack of unequivocal guidelines, early aggressive fluid management (at a rate of more than 1.5–2 times the maintenance rate of intravenous (IV) fluids) is recommended in children in the first 24 hr.
Oral feeding can be started as soon as tolerated even in the presence of systemic inflammation and before the amylase or lipase values have decreased.
If adequate oral feeding is not tolerated or the required calories cannot be achieved by oral feeding within 72 hr, enteral tube feeding is recommended.
Elemental and polymeric formulas are both appropriate in the management of AP.
Complete parenteral feeding is used as a second-line treatment in AP when enteral nutrition is not tolerable for the patient and additional nutrition is necessary.
Regardless of the severity of the pancreatitis or existing necrosis, routine use of prophylactic antibiotics is not recommended in AP.
In cases of systemic infectious complications, cholangitis or suspected infected pancreatic necrosis, antibiotic treatment is recommended.
In cases of severe cholangitis, ERCP should be done urgently within 24 hr. In other cases of cholangitis and/or obstruction, ERCP should be performed within 72 hr.
For uncomplicated biliary pancreatitis, cholecystectomy is recommended during the index admission if possible or, if not possible, within 30 days of the first admission for mild cholelithiasis-associated AP in children. Importantly, if cholecystectomy is not performed, the patient remains at risk of another episode of AP and complications of gallstones.
The most common medications associated with AP in children are valproic acid, mesalazine, thiopurines, and asparaginase
Acute recurrent pancreatitis (ARP) is defined by at least two acute attacks in a year or more than three in the patient's lifetime without any evidence of CP3.8 There must be complete resolution of pain (≥1 month pain-free interval between the diagnoses of AP, or complete normalization of serum pancreatic enzyme levels (amylase and lipase), before the subsequent episode of AP is diagnosed, along with complete resolution of pain symptoms, irrespective of a specific time interval between AP episodes. Studies suggest that children who had AP have a 10–35% chance of another attack.
Chronic pancreatitis (CP) is an irreversible inflammatory process that leads to changes in the pancreatic parenchyma and function. Documentation of characteristic histologic and morphologic alterations or decreased exocrine or endocrine pancreas function is needed to establish the diagnosis.
Reference
Parniczky 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.
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Pediatric Pancreatitis. This computed tomography (CT) scan of the abdomen in the region of the pancreas demonstrates a large well-marginated cystic structure that represents a pancreatic pseudocyst.
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Pediatric Pancreatitis. This real-time ultrasonogram of the abdomen, with attention to the right upper quadrant, reveals a loculated fluid collection in the hilum of the liver. This is compatible with a pancreatic pseudocyst. The differential diagnosis includes a large choledochal cyst.
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Pediatric Pancreatitis. Flow diagram for suspected acute pancreatitis . Etiologic factors and forms of acute pancreatitis and Ranson criteria are reviewed. ABG = arterial blood gas; Alk phos = alkaline phosphatase; ALT = alanine transaminase; AST = aspartate aminotransferase; BUN = blood urea nitrogen; CBC = complete blood cell count; Cr, creat = creatinine; ERCP = Endoscopic retrograde cholangiopancreatography; Gluc = glucose; lab = laboratory; LDH = lactate dehydrogenase; PO2 = partial pressure of oxygen; SBP = systolic blood pressure; and T bili = total bilirubin.
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Pediatric Pancreatitis. Flow diagram for mild pancreatitis. Favorable prognostic signs and medical management for acute pancreatitis, as well as studies used for acute pancreatitis. NPO = nothing by mouth; RUQ = right upper quadrant.
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Pediatric Pancreatitis. Prognostic indicators for severe pancreatitis and flow diagram for intensive care unit (ICU) management. BUN = blood urea nitrogen; CT = computed tomography; NG = nasogastric; and O2 = oxygen; paO2 = partial pressure of oxygen in arterial blood.
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Pediatric Pancreatitis. Flow diagram for diagnosis and treatment of necrotizing pancreatitis. CT = computed tomography; Tx = treatment.
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Pediatric Pancreatitis. Flow diagram for treatment of and studies used for pancreatic pseudocysts. ERCP = Endoscopic retrograde cholangiopancreatography
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Pediatric Pancreatitis. Flow diagram for management of idiopathic recurrent pancreatitis. Etiologies for acute pancreatitis. CBD = common bile duct; ERCP = Endoscopic retrograde cholangiopancreatography
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Pediatric Pancreatitis. Flow diagram for management of pancreatic abscess. Definition of an abscess.
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Pediatric Pancreatitis. A patient with acute gallstone pancreatitis underwent endoscopic retrograde cholangiopancreatography (ERCP). The cholangiogram showed no stones in the common bile duct and multiple small stones in the gallbladder. In this image, the pancreatogram shows narrowing of the pancreatic duct in the area of the genu, the result of extrinsic compression of the ductal system by inflammatory changes in the pancreas.
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Pediatric Pancreatitis. Abdominal computed tomography (CT) scan from the same patient as in the previous image showing pancreatic enlargement and peripancreatic fat stranding. The gallstones are not visible.
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Pediatric Pancreatitis. Pancreas divisum associated with minor papilla stenosis causing recurrent pancreatitis. Because pancreas divisum is relatively common in the general population, it is best regarded as a variant of normal anatomy and not necessarily as a cause of pancreatitis. In this case, note the bulbous contour of the duct adjacent to the cannula. This appearance has been termed a santorinicele. A dorsal duct outflow obstruction is the probable cause of pancreatitis when a santorinicele is present and associated with a minor papilla that accommodates only a guidewire.
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Pediatric Pancreatitis. Normal-appearing ventral pancreas in a patient with recurrent acute pancreatitis. The dorsal pancreas (not pictured) showed evidence of chronic pancreatitis.
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Pediatric Pancreatitis. Computed tomography (CT) scan of the abdomen in a child with traumatic pancreatitis. The fluid collection adjacent to the pancreas will become a pseudocyst. Note that the pancreas is lacerated, nearly cut in half, by the force of the abdominal trauma. Also, note the typical location of this injury in relation to the vertebral column.
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Pediatric Pancreatitis. Computed tomography (CT) scan of a young man who was referred after 2 weeks into his second bout of severe acute pancreatitis. Gravely ill, he had fever and leukocytosis as well as hypotension requiring pressors and respiratory distress requiring mechanical ventilation. His abdominal CT scan shows severe acute pancreatitis. A percutaneous drain was placed in the dominant fluid collection to establish drainage while he was given imipenem/cilastatin, which stabilized his condition.
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Pediatric Pancreatitis. Endoscopic retrograde cholangiopancreatography (ERCP) in the same patient as in the previous image excluded suppurative cholangitis and established the presence of annular pancreas divisum. The dorsal pancreatogram showed extravasation into the retroperitoneum, and a sphincterotomy was performed on the minor papilla. As shown in this radiographic film, a pigtailed nasopancreatic tube was then inserted into the dorsal duct and out into the retroperitoneal fluid collection. The other end of the tube was attached to the bulb suction and monitored every shift.
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Pediatric Pancreatitis. While percutaneous drains removed loculated fluid collections elsewhere in the abdomen in the same patient as in the previous image, the nasopancreatic tube contained the retroperitoneal fluid collection. One week later, the retroperitoneal fluid collection was much smaller, as shown in this radiographic film (the image is reversed in the horizontal direction). By this time, the patient was off pressors and was ready to be extubated.
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Pediatric Pancreatitis. In the same patient as in the previous image, 4 months later, after the pseudocyst was converted into a pseudocystogastrostomy using minimally invasive techniques, the pancreatogram revealed the more proximal pancreatic duct.
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Pediatric Pancreatitis. In the same patient as in the previous image, a guidewire was placed into the dorsal duct, crossed the stenotic area, and advanced into the proximal duct. A dilating catheter was then advanced over the wire to enlarge the stenosis. The duct was subsequently stented.
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Pediatric Pancreatitis. Six months after severe acute pancreatitis, the same patient as in the previous image remained symptom free and was living independently. As shown in this follow-up abdominal computed tomography (CT) scan, minimally invasive techniques were successful in removing the pockets of infection, restoring the integrity of the pancreatic ductal system. These techniques also preserved the endocrine function of the pancreas, and at the time of this follow-up, the patient had no evidence of diabetes mellitus.
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Pediatric Pancreatitis. Familial adenomatous polyposis syndrome in a patient with persistent pancreatitis due to a partially obstructing ampullary adenoma. The pancreatogram shown here reveals a very prominent ductal system. Because the patient had undergone several previous abdominal operations, she opted to have an endoscopic ampullectomy.
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Pediatric Pancreatitis. In this radiograph from the same patient as in the previous image, it can be seen that stents were placed into the biliary and pancreatic ductal systems following ampullectomy. The smoldering pancreatitis resolved within a week, the stents were subsequently removed, and the patient participated in an endoscopic surveillance program, with no recurrence at the time of this article's publication.
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Pediatric Pancreatitis. Recurrent pancreatitis associated with pancreas divisum in an elderly man. This pancreatogram of the dorsal duct shows a distal stenosis with upstream chronic pancreatitis. After the stenosis was dilated and stented, the patient's pain resolved, and he improved clinically during 1 year of stent exchanges on a quarterly basis. Follow-up computed tomography (CT) scans showed resolution of an inflammatory mass. Although ductal biopsies and cytology were repeatedly negative, pain and pancreatitis returned when the stents were removed. The patient developed duodenal outflow obstruction and was sent to surgery; a Whipple procedure revealed a periampullary adenocarcinoma (of the minor papilla).
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Pediatric Pancreatitis. Computed tomography (CT) scan of a patient initially seen for recurrent abdominal pain. An esophagogastroduodenoscopy (EGD) showed a submucosal nodule in the antrum, which prompted a referral to another center, with a request for endoscopic ultrasonography and polypectomy. Because the endoscopic ultrasonogram was indeterminate, a polypectomy was attempted. That evening, the patient developed progressively severe epigastric abdominal pain radiating to the back and presented to an emergency department. She had a leukocytosis and a mild elevation of her lipase and was admitted with a diagnosis of pancreatitis. This CT scan of her abdomen shows circumferential hypodense thickening of her antrum, with a normal-appearing pancreas. A small portion of pancreatic tissue was later identified as pancreatic rectitis in the pathology specimen.
