Pediatric Pancreatitis

Updated: Jan 19, 2023
Author: Andre Hebra, MD; Chief Editor: Carmen Cuffari, MD 

Overview

Practice Essentials

Pancreatitis, an uncommon condition in pediatric patients, is characterized by inflammation of the pancreas, clinical signs of epigastric abdominal pain, and elevated serum digestive enzymes. The disease is associated with significant morbidity and mortality.[1] Pancreatitis can be local or diffuse and is classified as acute, chronic, inherited, necrotic, or hemorrhagic. Occasionally, pancreatitis is complicated by the formation of a fibrous-walled cavity filled with pancreatic enzymes, termed a pseudocyst (see the image below).

Pediatric Pancreatitis. This computed tomography ( 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.

Signs and symptoms

Most commonly, a child with acute pancreatitis presents with the following:

  • Abdominal pain (87%)

  • Vomiting (64%)

  • Abdominal tenderness (77%)

  • Abdominal distention (18%)

Less common clinical signs include the following:

  • Fever

  • Tachycardia

  • Hypotension

  • Jaundice

  • Abdominal guarding

  • Rebound tenderness

  • Decreased bowel sounds

Acute hemorrhagic pancreatitis

Physical examination findings associated with hemorrhagic pancreatitis may include a bluish discoloration of the flanks (ie, Grey Turner sign) or periumbilical region (ie, Cullen sign) because of blood accumulation in the fascial planes of the abdomen. Additional signs include pleural effusions, hematemesis, melena, and coma.

Chronic pancreatitis

Patients with chronic pancreatitis have recurrent episodes of upper abdominal pain associated with varying degrees of pancreatic dysfunction.

Pancreatic pseudocysts

Children with pancreatic pseudocysts may present with localized abdominal pain and a palpable, tender epigastric mass or abdominal fullness. Additional symptoms include the following:

  • Jaundice

  • Chest pain

  • Nausea

  • Vomiting

  • Anorexia

  • Weight loss

  • Fever

  • Ascites

  • Gastrointestinal (GI) hemorrhage (rarely)

See Presentation for more detail.

Diagnosis

Laboratory studies

  • Elevated amylase levels

  • Elevated lipase levels

  • Coagulopathy

  • Leukocytosis

  • Hyperglycemia

  • Glucosuria

  • Hypocalcemia

  • Hyperbilirubinemia

  • Elevated gamma glutamyl transpeptidase

Imaging studies

  • Ultrasonography: Primary screening tool for evaluation of the pediatric pancreas, due to the absence of ionizing radiation and the ability to image without sedation

  • Computed tomography (CT) scanning

  • Endoscopic retrograde cholangiopancreatography (ERCP)

  • Magnetic resonance cholangiopancreatography (MRCP)

  • Radiography

Histologic features

  • Acute pancreatitis: Focal areas of fat necrosis are interspersed with areas of interstitial hemorrhage secondary to the destruction of blood vessels

  • Chronic pancreatitis: Features include Intraglandular fibrosis, acinar cell destruction, lymphocytic infiltration, and pancreatic duct obstruction

  • Pancreatic pseudocysts: Localized collections of pancreatic secretions walled off by granulation tissue and lacking a true epithelial lining

See Workup for more detail.

Management

Acute pancreatitis should resolve in 2-7 days with adequate resuscitation, with management being predominantly supportive through the use of intravenous hydration, pain control, and bowel rest. Parenteral nutrition may be required for prolonged episodes.

In cases of intractable vomiting or ileus, nasogastric suction is indicated to aid intestinal-pancreatic rest by eliminating gastric secretions in the duodenum, the most potent activator of pancreatic secretion.

Because most resolve spontaneously, acute pancreatic pseudocysts smaller than 5 cm in diameter are managed with observation for 4-6 weeks. (Pancreatic pseudocysts larger than 5 cm in diameter may require surgical intervention.)

Surgery

Most surgical interventions are used in patients with chronic or relapsing pancreatitis. However, operative management of chronic pancreatitis in children is controversial. Indications for operative intervention include unsuccessful conservative medical therapy, intractable pain, impaired nutrition, and narcotic addiction. Surgical options include the following:

  • Distal pancreatectomy with Roux-en-Y pancreaticojejunostomy (ie, Duval procedure)

  • Lateral pancreaticojejunostomy (ie, Puestow procedure)

  • ERCP sphincteroplasty

Chronic pancreatic pseudocysts (>3 mo) are best treated surgically. Surgical approaches for internal drainage, as follow, are largely determined by the anatomic location of the pseudocyst:

  • Cystogastrostomy: If the pseudocyst is adherent to the posterior wall of the stomach

  • Cystoduodenostomy: If the cyst is present in the head of the pancreas

  • Cystojejunostomy: For cysts that are not adherent to the stomach or duodenum

  • Distal pancreatectomy: Considered when the pseudocyst is in the tail of the gland

Some authors suggest that endoscopic treatment should be the first interventional choice for pancreatic pseudocysts; the success rate for such therapy is as high as 85%. Other surgical procedures can be reserved for cases that fail endoscopic treatment.

See Treatment and Medication for more detail.

Background

Pancreatitis, although uncommon during childhood, is associated with significant morbidity and mortality. This condition is characterized by inflammation of the pancreas, clinical signs of epigastric abdominal pain, and elevated serum digestive enzymes. Pancreatitis can be local or diffuse and is classified as acute, chronic, inherited, necrotic, or hemorrhagic. Occasionally, pancreatitis is complicated by the formation of a fibrous-walled cavity filled with pancreatic enzymes, termed a pseudocyst.

Although pancreatitis is uncommon in the pediatric population, clinicians evaluating patients with symptoms of abdominal pain should have a high index of suspicion for this condition. Pancreatitis originates with blockage or disruption of the collecting ducts and damage to the pancreatic acinar cells, which leads to activation and release of digestive enzymes. The activated enzymes autodigest the pancreatic parenchyma, causing inflammation and, potentially, necrosis. Localized collections of pancreatic secretions may become walled off by granulation tissue and form a pseudocyst either within the pancreatic tissue or immediately adjacent to it (see the images below).

Pediatric Pancreatitis. This computed tomography ( 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.
Pediatric Pancreatitis. This real-time ultrasonogr 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.

As a result of limited case reporting and underdiagnosis by physicians, the frequency and true incidence of pancreatitis in children is unknown, but is estimated to be 13 per 100,000 children.[2] Pseudocysts complicate acute pancreatitis in approximately 10-23% of cases. The incidence of pancreatic pseudocysts is greater than 50% when associated with traumatic injury to the abdomen.

See also the following:

  • Acute Pancreatitis

  • Chronic Pancreatitis

  • Emergent Management of Pancreatitis

Anatomy

The pancreas is divided up into a head, body, and tail, although no distinct anatomic borders indicate these divisions. Most of the pancreas is extraperitoneal, with just a portion of the tail coming through the mesenteric folds. The head is to the right of L2, the body overlies L1, and the tail rises up to the left of T12. The abdominal aorta and vena cava function to cushion the pancreas from injury against the vertebral bodies. However, with crushing or blunt abdominal trauma, the pancreas can be injured by compression against the vertebra.

Numerous congenital abnormalities of the pancreatic biliary system have been described. Pancreatic divisum, which occurs in 5-15% of the population, results from unsuccessful fusion of the embryonic ventral and dorsal pancreatic buds. As a result, the accessory duct of Santorini derived from the dorsal bud drains the majority of the pancreas. Because the accessory duct is smaller in caliber than the duct of Wirsung, inadequate pancreatic drainage may result in chronic pain and recurrent pancreatitis.

Etiology and Pathophysiology

Pediatric pancreatitis represents a diagnostic challenge for clinicians. Although most adult cases of pancreatitis are caused by alcohol abuse or gallstone disease, the etiology for pancreatitis in children is diverse.

The predominant causes include abdominal trauma (23%), anomalies of the pancreaticobiliary system (15%), multisystem disease (14%), drugs and toxins (12%), viral infections (10%), hereditary disorders (2%), and metabolic disorders (2%). In up to 25% of cases, the etiology of childhood pancreatitis is unknown. In the United States, trauma is responsible for 15-37% of cases.[3]

Common causes of pancreatitis are extensive but include blunt abdominal trauma (eg, motor vehicle collision, abuse, bicycle accident where the abdomen is compressed by the handlebars), systemic infection (eg, mumps, rubella, coxsackie virus B, cytomegalovirus [CMV], human immunodeficiency virus [HIV]), pancreaticobiliary malunion, congenital anomalies of the pancreatobiliary junction, pancreas divisum, congenital sphincter of Oddi abnormality, choledochal cysts, or choledocholithiasis.

Use of hyperalimentation, medications (eg, azathioprine, tetracycline, L-asparagine, valproic acid, steroids, and immunosuppressive agents), and metabolic abnormalities (eg, hypertriglyceridemia, hypercalcemia, cystic fibrosis) may also incite pancreatitis.

Hereditary pancreatitis in children, the second most common congenital pancreatic disorder following cystic fibrosis, is characterized by an alteration in the long arm of chromosome 7, which yields an aberrant trypsinogen protein that may induce autodigestion of the pancreas.

The specific inciting factors causing pancreatitis remain to be elucidated, including induction by primary acinar cell injury as a result of viral infections, drugs, ischemia, and direct trauma, as well as disruption of the ductal system and subsequent excretion of digestive enzymes from the acinar cells of the pancreas.

Normally, the acinar cells release inactive enzymes into collecting ducts, which then drain into the main or accessory pancreatic ducts emptying directly into the duodenal lumen. If obstruction or disruption of these ducts occurs, the pancreatic secretions are activated within the parenchyma of the pancreas and initiate autodigestion of the pancreatic tissue.

Interstitial edema is an early finding. Exacerbation of pancreatitis may result in pancreatic necrosis, blood vessel occlusion or disruption inciting hemorrhage, and systemic inflammatory response syndrome with multiorgan failure. Collections of pancreatic secretions often become walled off by granulation tissue to form a pseudocyst either within or adjacent to the pancreas. Predominantly, the pseudocyst is localized in the lesser sac behind the stomach. The stomach, duodenum, colon, small bowel, or omentum may abut or form part of the pseudocyst capsule.

Prognosis

In general, the prognosis of children with acute pancreatitis is excellent, although pseudocysts have been reported to complicate 10-23% of acute episodes. In addition, when associated with abdominal trauma, the frequency rate of pseudocyst identification is higher than 50%. Approximately 60% of pancreatic pseudocysts that are caused by blunt trauma require surgical intervention.

Complications

Although pseudocyst formation is an uncommon sequela of acute or chronic pancreatitis in children, complications of pancreatic pseudocysts include spontaneous rupture, hemorrhage, and infection. Pseudocysts can be medically managed with pancreatic rest or surgically by internal or external drainage. While under medical therapy, rupture is the major complication associated with pseudocysts larger than 10 cm.

 

Presentation

History and Physical Examination

Classically, pancreatitis in adults presents with midepigastric pain radiating to the back. In children, the presenting signs and symptoms can be quite varied. Most commonly, a child with acute pancreatitis presents with abdominal pain (94.9%), vomiting (60.4%), and nausea (58.2%).[4] Other, less common clinical signs include fever, tachycardia, hypotension, jaundice, abdominal guarding, rebound tenderness, and decreased bowel sounds. Eating may exacerbate the abdominal pain.

Acutely ill children may lie on their side with the hips and knees flexed. The pain typically increases in intensity for 24-48 hours. The clinical course for acute pancreatitis is variable. Often, children may require hospitalization for analgesia, bowel rest, and rehydration with fluid and electrolyte therapy.

Acute hemorrhagic pancreatitis

Acute hemorrhagic pancreatitis rarely occurs in children. This is a life-threatening condition with a mortality rate approaching 50% because of shock, systemic inflammatory response syndrome with multiple organ dysfunction, acute respiratory distress syndrome (ARDS), disseminated intravascular coagulation (DIC), massive gastrointestinal bleeding, and systemic or peritoneal infection.

Physical examination findings associated with hemorrhagic pancreatitis may include a bluish discoloration of the flanks (ie, Grey Turner sign) or periumbilical region (ie, Cullen sign) because of blood accumulation in the fascial planes of the abdomen. Additional signs include pleural effusions, hematemesis, melena, and coma.

Chronic pancreatitis

Chronic pancreatitis in children is associated with trauma, systemic disease, and pancreaticobiliary malformations, most commonly pancreatic divisum. In the United States, the most common cause of chronic relapsing pancreatitis in children is hereditary pancreatitis. Patients with this disease typically present with chronic abdominal pain that can be difficult to treat. These patients have recurrent episodes of upper abdominal pain associated with varying degrees of pancreatic dysfunction and have increased risk of developing pancreatic insufficiency, adenocarcinoma, and pancreatic pseudocysts.

Pancreatic pseudocysts

Children with pancreatic pseudocysts may present with localized abdominal pain and a palpable tender epigastric mass or abdominal fullness. Additional symptoms include jaundice, chest pain, nausea, vomiting, anorexia, weight loss, fever, ascites, and rarely, GI hemorrhage.

 

DDx

Diagnostic Considerations

Diagnosis of the specific cause of pancreatitis is important to elucidate, as there is a 9% recurrence rate, most of which are diagnosed with idiopathic recurrent pancreatitis or structural anomalies.

The most frequent cause of acute pancreatitis in pediatrics is related to medication administration. If a medication is suspected, it should be stopped immediately and other alternatives should be investigated. Etiologies related to trauma, systemic disease, or anatomic variants are important to determine and direct further medical or surgical interventions.

Differential Diagnoses

 

Workup

Approach Considerations

If pancreatitis is suspected, amylase and lipase levels should be measured, as they may support a clinical diagnosis. However, these laboratory test findings alone are not reliable or cost effective as a screening tool, and the magnitude of enzyme elevation does not correlate with the severity of pancreatic injury.[5]

Ultrasonography and computed tomography (CT) scanning are the preferred imaging modalities used to diagnose and follow the course of pancreatitis and pancreatic pseudocysts.

Laboratory Studies

Amylase levels

Elevated serum or urine amylase levels aid in the diagnosis of pancreatitis and peak 48 hours after onset, although 10-15% of patients with pancreatitis may have levels within the reference range. Serum amylase levels are typically elevated for as long as 4 days—although amylase levels can be elevated in patients with other abdominal conditions, the levels are typically not as high as those found in patients with pancreatitis.

Lipase levels

Serum lipase is more specific than amylase for acute pancreatitis, and typically, lipase levels remain elevated 8-14 days longer than amylase levels. Serum lipase levels can also be elevated in patients with other diseases or conditions; therefore, all laboratory results should be evaluated in the context of the clinical presentation.[6]

Other

Other laboratory abnormalities found in patients with pancreatitis may include coagulopathies, leukocytosis, hyperglycemia, glucosuria, hypocalcemia, hyperbilirubinemia, and elevated gamma glutamyl transpeptidase.

Urinary levels of trypsin activator peptide (TAP) may help determine the severity of the pancreatitis.

As many as 20% of children who present with acute pancreatitis develop severe disease that carries an increased morbidity, longer hospitalization, and need for aggressive medical management. A retrospective study showed that malnourished children were more likely to experience a protracted hospitalization independent of all the conventional biochemical parameters associated with an increased risk of disease severity in adults, including hypertriglyceridemia, ethnicity, or history of hepatobiliary disease.[7] In that study, most children with severe malnutrition had comorbid conditions, including cerebral palsy and encephalopathy, that could have masked the typical early clinical symptoms of acute pancreatitis.

One study provided a prognostic tool that may be used clinically to predict the severity of acute pancreatitis in children.[7] In that study, a predictive model based on a high serum lipase level (>19 the upper limit of normal) and a low albumin and white blood cell count was associated with an increased risk of morbidity. The authors concluded that the application of these three parameters in clinical practice may help pediatricians to identify those patients who are most at risk in developing severe pancreatitis and can thus help direct a more aggressive clinical management strategy.

Imaging Studies

Ultrasonography is the primary screening tool for evaluation of the pediatric pancreas, due to the absence of ionizing radiation and ability to image without sedation. Computed tomography (CT) scanning may be better suited for evaluation of chronic pancreatitis and its complications, pancreatic trauma, and neoplastic conditions and is often used to further evaluate abnormalities found on ultrasonography. Magnetic resonance imaging (MRI) is another modality to diagnose pancreatitis, providing tissue characterization and high-contrast imaging of the pancreatic duct without the use of invasive instrumentation or ionizing radiation.[8, 9]

Ultrasonography

Ultrasonography findings may include a focally or diffusely enlarged, hypoechoic, sonolucent, or edematous pancreas; dilated pancreatic ducts; a pancreatic mass; a fluid collection or peripancreatic fluid; an abscess; or a pseudocyst demonstrated as a well-defined, hypoechoic mass, which may be multilocular (see the image below).

Pediatric Pancreatitis. This real-time ultrasonogr 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.

CT scanning

CT scan findings include an enlarged gland with ill-defined margins; peripancreatic fluid; areas of decreased or enhanced density; or pseudocysts with a well-defined wall or capsule and central area of low attenuation (see the following images). CT scanning is a better modality for evaluating presence and extent of pancreatic necrosis and inflammation of peripancreatic fat.

Of note, findings on imaging studies initially appear normal in 20% of children with acute pancreatitis.

Pediatric Pancreatitis. Computed tomography (CT) s 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.
Pediatric Pancreatitis. Computed tomography (CT) s 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.
Pediatric Pancreatitis. Computed tomography (CT) s 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.

ERCP and MRCP

Endoscopic retrograde cholangiopancreatography (ERCP) is essential for evaluation of pancreatic and biliary anomalies. ERCP can aid in the diagnosis of various ductal abnormalities or obstructions and may serve as a therapeutic intervention (ie, sphincterotomy, stent placement) (see the images below). Magnetic resonance cholangiopancreatography (MRCP) is a noninvasive alternative to ERCP but lacks therapeutic capabilities.

Pediatric Pancreatitis. Familial adenomatous polyp 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.
Pediatric Pancreatitis. In this radiograph from th 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.
Pediatric Pancreatitis. Recurrent pancreatitis ass 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).

Radiography

Roentgenography may demonstrate nonspecific findings ranging from a distended loop of small intestine (ie, sentinel loop), calcifications, radio-opaque gallstones, dilatation of the transverse colon (ie, cutoff sign), ascites, peripancreatic extraluminal gas bubbles, ileus, left-sided basal pleural effusion, and blurring of the left psoas margin to pancreatic calcifications from chronic or recurrent pancreatitis.

Histologic Findings

Acute pancreatitis is characterized by enzymatic necrosis and inflammation of the pancreas. Focal areas of fat necrosis are interspersed with areas of interstitial hemorrhage secondary to destruction of blood vessels. In severe cases, large, blue-black hemorrhagic foci are interspersed with yellow-white chalky areas of fat necrosis.

Chronic pancreatitis is characterized by irreversible destruction of the pancreatic parenchyma and subsequent replacement with fibrous tissue. Histologic features include intraglandular fibrosis, acinar cell destruction, lymphocytic infiltration, and pancreatic duct obstruction. The pancreatic ducts are dilated and obstructed with protein plugs in their lumens. Grossly, the gland is hard.

Pancreatic pseudocysts are localized collections of pancreatic secretions walled off by granulation tissue that lack a true epithelial lining. The stomach, duodenum, small bowel, colon, or omentum may abut or form part of the pseudocyst capsule.

 

Treatment

Approach Considerations

Cases of uncomplicated acute pancreatitis usually resolve within 2-4 days. The management of acute pancreatitis is predominately supportive medical therapy, with intravenous hydration, pain control, and bowel rest. Parenteral nutrition may be required for prolonged episodes.

Nutrition has an important role in the management of patients with mild acute pancreatitis. A database analysis of all children admitted with mild acute pancreatitis from a major institution failed to show any relationship between pain severity among those patients who received either a low fat or regular diet compared to those patients who were restricted from oral feeding.[10] Moreover, most patients tolerated their nutrition by mouth when compared with those children who received nutritional therapy either from a nasogastric or jejunal feeding tube.

Surgical management is used to address complications of pancreatitis, including hemorrhage, necrosis, ductal fistulae, and pseudocysts. Surgical management of pancreatic pseudocysts is highly successful. Recurrence rates and mortality rates are low. Internal drainage is associated with lower recurrence rates compared to percutaneous or endoscopic drainage. Transendoscopic and percutaneous drainage of pancreatic pseudocysts have been predominately performed in the adult population, and further investigation and comparison of these techniques in children is warranted to determine the optimal management of this disease.

Sphincteroplasty is contraindicated in patients with evidence of intrapancreatic ductal obstruction. Pancreatic pseudocysts with associated major ductal disruption or a transected pancreas require operative therapy with cyst enterostomy. Definitive management with subtotal or total pancreatectomy is associated with considerable morbidity and mortality due to loss of both endocrine and exocrine functions of the pancreas. Generally, surgery is not indicated in children; however, it may be considered in cases of intractable pain and diffuse parenchymal damage without ductal dilation.

The use of total pancreatectomy with islet cell transplantation is undergoing evaluation for the treatment of chronic abdominal pain in children with chronic pancreatitis. Islet cell transplantation allows a patient to be treated for the pain of pancreatitis without the very serious adverse effects of a total pancreatectomy, including "brittle diabetes," which occurs when a person's blood glucose level often swings quickly from high to low and from low to high.[11]

Consultations

Treatment of pediatric patients with chronic pancreatitis requires the collaborative efforts of multispecialty teams that include gastroenterologists, surgeons, pharmacologists, nutritionists, child life specialists, psychologists, and psychiatrists.

Medical Care

The goal of medical management of acute pancreatitis is to achieve adequate rehydration, analgesia, and pancreatic rest and to restore normal metabolic homeostasis. In patients with severe pancreatitis, oral intake is restricted and parenteral nutrition is initiated within 3 days to prevent catabolism.

In cases of intractable vomiting or ileus, nasogastric suction is indicated to help intestinal-pancreatic rest by eliminating gastric secretions in the duodenum, the most potent activator of pancreatic secretion. Fluid electrolyte and mineral imbalances should be corrected urgently. Antibiotic therapy is indicated for systemic infections or sepsis.

Acute pancreatitis should resolve in 2-7 days with adequate resuscitation. In the setting of chronic relapsing pancreatitis, pancreatic enzyme supplementation, insulin, and elemental or low-fat diets are useful adjuncts to maximize nutritional status.[12]

For alleviation of pain, meperidine is preferred over morphine because of its decreased risk of ampullary spasm.

Preoperative studies with ultrasonography and computed tomography (CT) scanning, the preferred imaging modalities used to diagnosis and follow the course of pancreatitis and pancreatic pseudocysts, are important to assess the character and size of the pseudocyst. Endoscopic retrograde cholangiopancreatography (ERCP) is essential to assess various ductal abnormalities or pseudocyst communication with the pancreatic duct to determine definitive operative therapy.

Surgical Care

Surgical management of acute pancreatitis is rarely required. Surgical intervention is only needed if the symptoms are severe and prolonged or complicated by necrosis or abscess formation that requires debridement. Peritoneal lavage has been used in adults in an effort to reduce the incidence of secondary infection; however, this has not been through trials with children to test its efficacy. If underlying pancreaticobiliary disease is present, surgical intervention is required for cure.

Most surgical interventions are used in patients with chronic or relapsing pancreatitis. The goal of surgery in this instance is to alleviate pain and preserve the exocrine and endocrine functions of the pancreas. Surgical therapies include longitudinal pancreaticojejunostomy, distal pancreatectomy with Roux-en-Y pancreaticojejunostomy, decompression of pancreatic ducts, repair of pancreatic divisum, and sphincteroplasty.

However, operative management of chronic pancreatitis in children is controversial. Indications for operative intervention include unsuccessful conservative medical therapy, intractable pain, impaired nutrition, and narcotic addiction. Surgical options include distal pancreatectomy with Roux-en-Y pancreaticojejunostomy (ie, Duval procedure), lateral pancreaticojejunostomy (ie, Puestow procedure), or ERCP sphincteroplasty. A few pediatric patients with chronic pancreatitis and chronic abdominal pain were successfully treated with total pancreatectomy and islet cell transplantation.[11, 13, 14]

Surgical intervention is also indicated for the management of congenital anatomic defects (eg, pancreatic divisum) and other complications associated with acute pancreatitis (eg, pancreatic ascites, intra-abdominal abscess collections, pancreatic pseudocyst).

Pancreatic pseudocysts

Acute pancreatic pseudocysts smaller than 5 cm in diameter are managed with observation for 4-6 weeks because most resolve spontaneously. Pancreatic pseudocysts larger than 5 cm in diameter may require surgical intervention; however, conservative therapy is required for approximately 4-6 weeks to allow the cyst wall to mature. Results from a study by Ford et al indicate that pancreatic pseudocysts larger than 10 cm in diameter in children are associated with increased risk for spontaneous rupture and, thus, require aggressive monitoring.[15]

Chronic pancreatic pseudocysts (>3 mo) are best treated by surgical interventions. Ultrasonography-guided or CT-guided percutaneous drainage,[16] endoscopic drainage, and internal drainage via cyst gastrostomy or enterostomy have been used with success. Surgical approaches for internal drainage are largely determined by the anatomic location of the pseudocyst. If the pseudocyst is adherent to the posterior wall of the stomach, cystogastrostomy is performed. If the cyst is present in the head of the pancreas, cystoduodenostomy is considered. For other cysts not adherent to the stomach or duodenum, cystojejunostomy is preferred. Distal pancreatectomy is considered when the pseudocyst is in the tail of the gland.

Managing pancreatic pseudocysts with endoscopic treatment has been an increasing trend. It has been demonstrated that, in skilled hands, endoscopic treatment is safe and effective (for short-term and long-term treatment). Some authors suggest that endoscopic treatment should be the first interventional choice. The success rate is as high as 85%. Surgical treatment can be reserved for those cases that fail endoscopic treatment.

Pancreatic ductal disruption or compromise

Surgery for pancreatic ductal disruption or compromise (ie, acute traumatic pancreatitis with ductal injury) is indicated after medical failure. Endoscopic retrograde cholangiopancreatography (ERCP) or intraoperative pancreatic ductography is invaluable in identifying and determining the site of ductal disruption and directs surgical decision-making to the most appropriate operative procedure.[17, 18, 19]

 

Guidelines

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.

 

Medication

Medication Summary

No medications are used to treat acute pancreatitis specifically. Therapy is primarily supportive to relieve pain and minimize complications. Such therapy involves intravenous fluid hydration, analgesics, antibiotics (in severe pancreatitis), and treatment of metabolic complications (eg, hyperglycemia, hypocalcemia).

Analgesics

Class Summary

Analgesic agents are used to reduce pain and inflammation, which is essential to quality patient care. These medications ensure patient comfort, promote pulmonary toilet, and have sedating properties, which are beneficial for patients who have sustained trauma or have painful lesions.

Propoxyphene products were withdrawn from the United States market on November 19th, 2010. The withdrawal was based on new data showing QT prolongation at therapeutic doses. For more information, see the FDA MedWatch safety information.

Acetaminophen (Tylenol, Feverall, Aspirin Free Anacin)

Acetaminophen is a peripherally acting drug of choice (DOC) for mild pain and elevation of body temperature.

Tramadol (Ultram, Ryzolt, Rybix)

Tramadol is a centrally acting analgesic indicated for moderately severe pain. This agent inhibits ascending pain pathways, altering the perception of and response to pain. Tramadol also inhibits reuptake of norepinephrine and serotonin.

Meperidine (Demerol)

Meperidine is a synthetic opioid narcotic analgesic indicated for the relief of severe pain. This analgesic has multiple actions similar to those of morphine. However, meperidine may produce less constipation, smooth muscle spasm, and depression of cough reflex than similar analgesic doses of morphine.

Antibiotics

Class Summary

Imipenem and cilastatin are used in combination for the treatment of multiple-organism infections in which other agents do not have wide-spectrum coverage or are contraindicated because of potential for toxicity. These agents are generally administered in a 1:1 combination.

The combination is a thienamycin derivative with greater potency and broader antimicrobial spectrum than other beta-lactam antibiotics. Cilastatin inhibits dehydropeptidase activity and reduces cilastatin metabolism.

Imipenem and cilastatin (Primaxin)

Imipenem and cilastatin are used in combination for the treatment of multiple-organism infections in which other agents do not have wide-spectrum coverage or are contraindicated because of potential for toxicity. These agents are generally administered in a 1:1 combination.

The combination is a thienamycin derivative with greater potency and broader antimicrobial spectrum than other beta-lactam antibiotics. Cilastatin inhibits dehydropeptidase activity and reduces imipenem metabolism.

Ampicillin

Ampicillin has bactericidal activity against susceptible organisms and is used as an alternative agent to amoxicillin when a patient is unable to take medication orally.

Ceftriaxone (Rocephin)

Ceftriaxone is a third-generation cephalosporin with broad-spectrum gram-negative activity; lower efficacy against gram-positive organisms; and higher efficacy against resistant organisms. This agent acts by arresting bacterial growth through binding to one or more penicillin-binding proteins.