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 (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:
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Abdominal pain (87%)
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Vomiting (64%)
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Abdominal tenderness (77%)
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Abdominal distention (18%)
Less common clinical signs include the following:
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Fever
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Tachycardia
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Hypotension
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Jaundice
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Abdominal guarding
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Rebound tenderness
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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:
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Jaundice
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Chest pain
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Nausea
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Vomiting
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Anorexia
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Weight loss
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Fever
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Ascites
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Gastrointestinal (GI) hemorrhage (rarely)
See Presentation for more detail.
Diagnosis
Laboratory studies
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Elevated amylase levels
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Elevated lipase levels
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Coagulopathy
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Leukocytosis
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Hyperglycemia
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Glucosuria
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Hypocalcemia
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Hyperbilirubinemia
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Elevated gamma glutamyl transpeptidase
Imaging studies
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Ultrasonography: Primary screening tool for evaluation of the pediatric pancreas, due to the absence of ionizing radiation and the ability to image without sedation
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Computed tomography (CT) scanning
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Endoscopic retrograde cholangiopancreatography (ERCP)
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Magnetic resonance cholangiopancreatography (MRCP)
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Radiography
Histologic features
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Acute pancreatitis: Focal areas of fat necrosis are interspersed with areas of interstitial hemorrhage secondary to the destruction of blood vessels
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Chronic pancreatitis: Features include Intraglandular fibrosis, acinar cell destruction, lymphocytic infiltration, and pancreatic duct obstruction
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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:
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Distal pancreatectomy with Roux-en-Y pancreaticojejunostomy (ie, Duval procedure)
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Lateral pancreaticojejunostomy (ie, Puestow procedure)
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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:
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Cystogastrostomy: If the pseudocyst is adherent to the posterior wall of the stomach
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Cystoduodenostomy: If the cyst is present in the head of the pancreas
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Cystojejunostomy: For cysts that are not adherent to the stomach or duodenum
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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 (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 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:
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.
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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.
