Basics
Description
- Inflammation within the pancreas characterized by 3 phases: early trypsin activation within acinar cells; followed by surrounding intrapancreatic inflammation; and finally, extrapancreatic inflammation with systemic inflammatory responses
- Classified into acute and chronic
- Acute pancreatitis (AP)
- Variable presentation, however, most often characterized by acute onset of abdominal pain, nausea, and vomiting with elevation of pancreatic enzymes
- Nonverbal children may present with irritability; infants may present with lethargy and fever.
- Often self-limited, with reversible changes, but can progress if not appropriately managed
- Severe AP is rare in children; however, a high suspicion should always be maintained, as severe disease can progress rapidly and result in significant morbidity and mortality.
- Chronic pancreatitis (CP)
- Characterized by irreversible morphologic changes and fibrotic replacement of the pancreatic parenchyma
- Clinically characterized by recurrent abdominal pain or evidence of exocrine and/or endocrine insufficiency
- Often the result of a persistent and continued pancreatic inflammation secondary to acute attacks
Etiology
- Biliary tract disease
- Medications
- L-asparaginase, azathioprine/6-MP, mesalamine, sulfonamides, thiazides, furosemide, tetracyclines, valproic acid, corticosteroids, estrogens, procainamide, ethacrynic acid, and others
- Toxins
- Alcohol, organophosphates, scorpion poison, snake poison
- Trauma
- Bicycle handle injuries
- Motor vehicle collisions
- Child abuse
- Postoperative
- Endoscopic retrograde cholangiopancreatography (ERCP)
- Systemic disease
- Shock/hypoxemia/SEPSIS
- Inflammatory bowel disease (IBD)
- Cystic fibrosis
- Idiopathic
- Less common
- Infections
- Bacterial: typhoid, mycoplasma
- Viral: measles, mumps, Epstein-Barr virus, coxsackie B, rubella, influenza, echovirus, hepatitis A and B
- Parasites: Ascaris lumbricoides, Echinococcus granulosus, Cryptosporidium parvum, Plasmodium falciparum
- Metabolic diseases:
- Hyperlipidemia
- Hypercalcemia
- Diabetic ketoacidosis
- Uremia
- Inborn errors of metabolism
- Systemic diseases:
- Hemolytic uremic syndrome
- Celiac disease
- Diabetes mellitus
- Vasculitis: systemic lupus erythematosus (SLE), Henoch-Sch Άnlein purpura, Kawasaki disease
- Rare causes:
- Autoimmune pancreatitis: Rare condition divided into 2 subtypes.
- Type I often grouped with IgG4-related disease and systemic manifestations.
- Type II does not have IgG4 association, however, is more common in younger patients and associated with IBD.
- Congenital anomalies:
- Pancreatic divisum
- Annular pancreas
- Anomalous pancreaticobiliary junction
- Biliary tract malformations
- Duplication cyst of the duodenum/gastropancreatic/common bile duct
Diagnosis
History
- Pain
- Onset, location, and severity can be variable in children.
- Aggravated by food intake
- Nonverbal patients (i.e., younger age, developmental delay) may present with increased irritability.
- Vomiting
- May or may not always be present
- May be bilious
- May present as feeding intolerance
- Trauma
- Even trivial abdominal trauma should be a red flag.
- Evaluate for evidence of child abuse.
- Family history
- Hereditary pancreatitis
- Hypertriglyceridemia (I, IV, or V)
- CFTR mutations/FH of CF
- Prior history of, or risk factors for, cholelithiasis
- Toxic exposures (i.e., EtOH, pesticides)
- Review of systems:
- Associated fever may suggest infectious etiology.
- Diminished urine output raises concern for third-space losses.
- Shortness of breath raises concern for pulmonary involvement (i.e., effusions).
Physical Exam
- General exam
- Growth parameters (weight and height), vital signs, capillary refill, pulse oximetry, pallor, jaundice, edema, and clubbing
- Abnormal vital signs (heart rate, respiratory rate, and blood pressure) can be indicative of the systemic inflammatory response syndrome (SIRS), a poor prognostic sign.
- Clubbing can be an indicator of cystic fibrosis.
- GI
- Mouth: Presence of aphthous lesions raises possibility of Crohn disease.
- Inspection: abdominal distention or flank fullness (ascites or pseudopancreatic cyst); bluish discoloration of the flanks (Grey Turner sign) and periumbilical region (Cullen sign) in hemorrhagic pancreatitis
- Palpation: for liver, gall bladder, spleen, and masses. Patients will have guarding, tenderness, and rebound tenderness, especially in the epigastric region and/or upper abdomen; palpable mass could be a pancreatic pseudocyst.
- Percussion: dullness and fluid thrill consistent with ascites
- Auscultation: bowel sounds decreased in ascites or absent in paralytic ileus
- Rectal exam
- Perianal region for skin tags, fistulas, abscesses, or healed scars, which could be indicative of IBD; perirectal exam for mass, melena, or occult blood
- Hematochezia can be suggestive of IBD.
- GU
- Assess urinary output.
- High specific gravity suggestive of reduced intravascular volume, result of third-space losses
- Respiratory system
- Pleural effusion and acute respiratory distress syndrome (ARDS)
- Diffuse respiratory findings could be indicative of cystic fibrosis.
- CNS
Diagnostic Tests & Interpretation
Lab
- CBC
- Hemoglobin may be decreased in hemorrhagic pancreatitis.
- Leukocytosis may be present in infectious pancreatitis.
- Hemoconcentration: elevated HCT
- Basic metabolic panel
- Hemoconcentration due to third-space losses and intravascular depletion (elevated BUN, Cr)
- Calcium may be elevated (etiology) or decreased (sequelae).
- Glucose may be transiently elevated.
- Bicarbonate may be low secondary to acidosis.
- Liver function tests:
- Elevated transaminase levels suggest biliary cause.
- Elevated bilirubin level/GGTP/Alk Phos suggestive of gallstone pancreatitis
- Amylase level
- 3-fold elevation of amylase levels increases specificity for the diagnosis of pancreatitis.
- Starts rising 2 " 12 hours after the insult and remains elevated for 3 " 5 days
- Degree of elevation does not have any correlation to the severity or the course of the illness.
- Other causes of elevated amylase levels include bowel obstruction, acute appendicitis, biliary obstruction, salivary duct obstruction, diabetic ketoacidosis, cystic fibrosis, pneumonia, salpingitis, ruptured ectopic pregnancy, ovarian cyst, cerebral trauma, burns, renal failure, and macroamylasemia.
- Lipase level
- Lipase levels are more specific than amylase for the diagnosis of pancreatitis.
- Starts rising 4 " 8 hours after the insult and remains elevated for 8 " 14 days
- 3-fold increase in the level is very sensitive and specific for pancreatitis.
- Levels do not correlate with severity or with clinical outcome.
- Other causes of elevated lipase levels include intestinal perforation, intestinal obstruction, appendicitis, mesenteric infarction, cholecystitis, diabetic ketoacidosis, renal failure, and macrolipasemia.
- Urinalysis
- Urine specific gravity is a simple indicator of intravascular volume.
- Elevated value can suggest third-space losses, a harbinger for severe disease.
Imaging
- Abdominal x-rays:
- Sentinel loop: distended small intestinal loop near the pancreas
- Colon cutoff sign: absence of gas shadow in the colon distal to the transverse colon
- Multiple fluid levels in paralytic ileus
- Calcification or stones in pancreas or gallbladder
- Diffuse haziness: ascites
- Chest x-ray
- Pleural effusion or ARDS
- Diaphragmatic involvement
- US abdomen
- Best initial test
- Will demonstrate pancreatic size; echogenicity; associated fat stranding; ductal diameter/disruption; and calcifications, cholelithiasis, CBD dilation, ascites, and free fluid within abdomen
- Limited by obesity and bowel gas
- Endoscopic US (EUS) is more useful than the transabdominal US study but is difficult in children; used if pancreatic biopsy is indicated
- CT scan
- May be used if history of trauma to look at extent of injury to pancreas and other intra-abdominal structures
- Best to show evidence of pancreatic necrosis with AP from any cause, however, not sensitive within the first 48 hours
- Reveals pathology in the pancreaticobiliary system in most instances
- Involves exposure to radiation
- Magnetic resonance cholangiopancreatography (MRCP)
- Good for ductal visualization (especially if secretin stimulated)
- Can reveal anatomic abnormalities/obstructive lesions, that is, pancreatic divisum
- Can mitigate need for ERCP, unless there is concern for choledocholithiasis +/ ’ ascending cholangitis
- Good for distinguishing pseudocyst versus walled-off necrosis
- No exposure to radiation
- ERCP
- Indicated in persistent/CP for delineation of pancreatic ducts and for therapeutic interventions (i.e., sphincterotomy or stent placement)
- Risk of post-ERCP pancreatitis in 10 " 20% of cases
- In adults, administration of rectal indomethacin has been shown to reduce this risk.
Treatment
Fluid Resuscitation
- Aggressive volume resuscitation with isotonic solutions is the cornerstone of therapy for AP.
- Under-resuscitation is associated with increased risk of mortality from AP.
- Targeted approach involving bolus (20 mL/kg), followed by continuous infusion at 1.5 " 2 maintenance (contraindicated if presence of fluid-sensitive cardiac disease) with serial monitoring q6 " 8h
Medication
- Antibiotics
- No evidence to support the routine use of antibiotics in patients with acute necrotizing pancreatitis unless:
- Sepsis is suspected.
- An extrapancreatic infection (bacteremia, pneumonia, UTI) is present.
- Patient is on chemotherapy and/or is neutropenic.
- Pain management
- Effective analgesia should be a priority.
- Combination of intermittent IV narcotics +/ ’ PCA
Additional Treatment
Nutrition
- Initially, patient should remain NPO for pancreatic rest.
- New evidence suggests early feeding may not be beneficial.
- Postpyloric feeds can be considered if initial trial of enteral feeding fails.
- If prolonged NPO course and failure of postpyloric feeds, consider parenteral nutrition.
Surgery/Other Procedures
- If gallstone impaction with choledocholithiasis, should consider ERCP + sphincterotomy + stent
- Severe third-spacing can lead to abdominal compartment syndrome, necessitating surgical decompression.
- Development of walled-off necrosis with persistent pain may benefit from necrocystectomy (endoscopic or surgical).
Ongoing Care
Prognosis
AP is usually a self-limiting disorder in children.
Complications
- Pancreatic edema
- Peripancreatic fat necrosis
- Acute pancreatic fluid collections
- Pancreatic necrosis
- Pancreatic pseudocyst or walled-off necrosis
- Pancreatic ductal strictures
- Pancreatic ductal dilatation
- Systemic complications:
- Shock and multiorgan failure
- GI and hepatobiliary
- Paralytic ileus
- Ascites, peritonitis
- Stress ulcer
- Intestinal hemorrhage
- Portal vein thrombosis/splenic vein thrombosis/obstruction
- Bile duct obstruction
- Pulmonary
- Atelectasis, pleural effusion, pneumonitis, ARDS
- Cardiovascular
- Hypotension/circulatory collapse
- Pericarditis/pericardial effusion
- EKG changes
- Sudden death
Additional Reading
- Bai HX, Lowe ME, Husain SZ. What have we learned about acute pancreatitis in children? J J Pediatr Gastroenterol Nutr. 2011;52(3):262 " 270. [View Abstract]
- Banks PA, Bollen TL, Dervenis C, et al. Classification of acute pancreatitis " 2012: revision of the Atlanta classification and definitions by international consensus. Gut. 2012;62(1):102 " 111. [View Abstract]
- Forsmark CE, Baillie J; AGA Institute Clinical Practice and Economics Committee; AGA Institute Governing Board. AGA Institute technical review on acute pancreatitis. Gastroenterology. 2007;132(5):2022 " 2044. [View Abstract]
- Tenner S, Baillie J, DeWitt J, et al. American College of Gastroenterology guideline: management of acute pancreatitis. Am J Gastroenterol. 2013;108(9):1400 " 1415. [View Abstract]
Codes
ICD09
- 577.0 Acute pancreatitis
- 577.1 Chronic pancreatitis
ICD10
- K85.9 Acute pancreatitis, unspecified
- K86.1 Other chronic pancreatitis
- K85.8 Other acute pancreatitis
- K85.0 Idiopathic acute pancreatitis
SNOMED
- 75694006 Pancreatitis (disorder)
- 197456007 Acute pancreatitis (disorder)
- 235494005 Chronic pancreatitis (disorder)
- 39205007 Infectious pancreatitis (disorder)