Pediatric Pancreatitis

Updated: Aug 15, 2016
  • Author: Andre Hebra, MD; Chief Editor: Carmen Cuffari, MD  more...
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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).

This computed tomography (CT) scan of the abdomen 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 Clinical 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.

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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).

This computed tomography (CT) scan of the abdomen 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.
This real-time ultrasonogram of the abdomen, with 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. 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
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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.

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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. [2]

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.

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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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