Overview
Definition:
Acute pancreatitis in children and adolescents characterized by severe inflammation of the pancreas, triggered by markedly elevated serum triglyceride levels, typically >1000 mg/dL (11.3 mmol/L)
This leads to the hydrolysis of triglycerides into free fatty acids, causing direct acinar cell injury and microvascular damage.
Epidemiology:
While less common than in adults, hypertriglyceridemia-induced pancreatitis (HTGP) is a significant cause of acute pancreatitis in pediatric populations, accounting for up to 20% of cases
Predominantly seen in adolescents, often associated with underlying genetic dyslipidemias or secondary causes like obesity and poor dietary habits
Exact incidence data is limited but is a critical consideration in patients with severe hypertriglyceridemia.
Clinical Significance:
HTGP is a medical emergency with potentially severe morbidity and mortality
Early recognition and aggressive management are crucial to prevent systemic complications such as organ failure (respiratory, renal), disseminated intravascular coagulation (DIC), and chronic pancreatitis
Understanding this entity is vital for pediatricians and residents preparing for DNB and NEET SS exams, as it requires prompt diagnosis and multidisciplinary care.
Clinical Presentation
Symptoms:
Sudden onset of severe, constant, epigastric abdominal pain radiating to the back
Nausea and vomiting are common
Fever may be present
In younger children, symptoms might be less specific, presenting as irritability, abdominal distension, or poor feeding
Jaundice can occur if there is biliary involvement or compression.
Signs:
Tenderness in the epigastrium is the hallmark finding
Guarding and rebound tenderness may indicate peritonitis
Tachycardia and hypotension suggest hypovolemia or systemic inflammatory response syndrome (SIRS)
Signs of shock, such as cool extremities and altered mental status, indicate severe disease
Xanthomas (especially eruptive xanthomas) may be visible in cases of severe, chronic hyperlipidemia
Grey Turner's sign (flank ecchymosis) and Cullen's sign (periumbilical ecchymosis) are rare but indicate retroperitoneal hemorrhage.
Diagnostic Criteria:
The diagnosis of acute pancreatitis is based on the presence of at least two of the following three features: 1
Characteristic abdominal pain (epigastric, sudden onset, severe, radiating to the back)
2
Serum amylase and/or lipase levels at least three times the upper limit of normal
3
Characteristic findings of acute pancreatitis on cross-sectional abdominal imaging (contrast-enhanced CT, MRI, or transabdominal ultrasound)
For HTGP, extremely elevated triglyceride levels (>1000 mg/dL) are a critical diagnostic clue alongside the pancreatitis findings.
Diagnostic Approach
History Taking:
Detailed family history for dyslipidemias, diabetes, cardiovascular disease, or pancreatitis
Dietary history including intake of fats, sugars, and alcohol
Review of medications that can cause hypertriglyceridemia (e.g., estrogens, corticosteroids, certain antiretrovirals)
History of recurrent abdominal pain, unexplained weight gain, or obesity
Assess for symptoms of genetic dyslipidemias like eruptive xanthomas.
Physical Examination:
Abdominal examination focusing on tenderness, guarding, rigidity, and bowel sounds
Assess for signs of systemic involvement: vital signs (heart rate, blood pressure, respiratory rate, temperature), oxygen saturation, and neurological status
Examine skin for eruptive xanthomas, jaundice, or signs of bleeding (Grey Turner's, Cullen's).
Investigations:
Serum triglycerides: Critical for diagnosis, levels >1000 mg/dL strongly suggest HTGP
Serum amylase and lipase: Elevated (typically >3x ULN)
Lipase is more specific and has a longer half-life
Complete blood count (CBC): Leukocytosis is common
Liver function tests (LFTs): May show elevated bilirubin, AST, ALT, alkaline phosphatase, and GGT if biliary obstruction or hepatic steatosis is present
Serum calcium: Hypocalcemia can occur due to saponification of fat in the pancreas
Renal function tests: Monitor for acute kidney injury
Blood glucose: Hyperglycemia is common
Arterial blood gas (ABG): Assess for metabolic acidosis and oxygenation
Lipid profile: To assess other lipid abnormalities and guide long-term management
Imaging: Abdominal ultrasound is often the initial imaging modality to rule out gallstones and assess for pancreatic inflammation
Contrast-enhanced CT scan of the abdomen is highly sensitive for detecting pancreatic inflammation, necrosis, and complications
it is typically performed 48-72 hours after symptom onset if the diagnosis is uncertain or severe disease is suspected
MRI/MRCP can be useful for evaluating biliary tree and pancreatic ductal anatomy.
Differential Diagnosis:
Other causes of acute pancreatitis in children: Gallstone pancreatitis, viral pancreatitis (e.g., mumps, coxsackievirus), trauma-induced pancreatitis, drug-induced pancreatitis, idiopathic pancreatitis, autoimmune pancreatitis, hypercalcemia-induced pancreatitis, infection-related pancreatitis (e.g., enterovirus, Mycoplasma), congenital anomalies of the pancreas
Differentiating HTGP relies on the presence of extremely high triglyceride levels.
Management
Initial Management:
Immediate resuscitation and stabilization are paramount
This includes intravenous fluid resuscitation (e.g., Lactated Ringer's solution) to maintain adequate hydration and perfusion
Pain management with intravenous analgesics (e.g., morphine, hydromorphone)
Nasogastric tube insertion for decompression if vomiting is persistent or ileus is present
Strict NPO (nil per os) status initially to rest the pancreas
Monitoring of vital signs, urine output, and fluid balance is critical.
Medical Management:
The cornerstone of HTGP management is rapid reduction of triglyceride levels
This can be achieved through: 1
Intravenous Insulin Infusion: Even in the absence of hyperglycemia, insulin infusion (e.g., 0.1-0.2 units/kg/hr) activates lipoprotein lipase, promoting triglyceride hydrolysis
2
Plasmapheresis or Plasma Exchange: Used in severe cases or when triglyceride levels do not rapidly decline, effectively removing triglyceride-rich lipoproteins
3
Anticoagulation: Heparin may be considered to enhance lipoprotein lipase activity, though its role is debated and should be used cautiously
4
Fibrates and Omega-3 Fatty Acids: For long-term management of hypertriglyceridemia, but not for acute management
Consider medications that can exacerbate hypertriglyceridemia for discontinuation if appropriate.
Surgical Management:
Surgery is generally reserved for managing complications of pancreatitis, such as infected pancreatic necrosis or pseudocysts, rather than for the pancreatitis itself
Indications include signs of infection (fever, leukocytosis, positive blood cultures) in necrotic areas, or symptomatic, expanding pseudocysts
Minimally invasive techniques like percutaneous drainage or endoscopic necrosectomy are preferred over open surgery when possible.
Supportive Care:
Nutritional support should be initiated cautiously once pain and nausea subside
Enteral feeding, preferably via a nasojejunal tube, is preferred over parenteral nutrition (PN) to preserve gut integrity and reduce the risk of infectious complications
Close monitoring for complications such as SIRS, organ failure (renal, respiratory), and infection
Electrolyte and glucose monitoring and correction
Psychological support for the patient and family.
Complications
Early Complications:
Systemic inflammatory response syndrome (SIRS) leading to multi-organ dysfunction syndrome (MODS), including acute respiratory distress syndrome (ARDS), acute kidney injury (AKI), and cardiovascular instability
Pancreatic necrosis (both sterile and infected), pancreatic pseudocysts, pancreatic abscesses, hemorrhage, and pseudoaneurysm formation
Disseminated intravascular coagulation (DIC).
Late Complications:
Chronic pancreatitis with exocrine and endocrine insufficiency (diabetes mellitus)
Pancreatic pseudocysts that persist and cause symptoms
Increased risk of pancreatic cancer with recurrent or chronic pancreatitis
Malnutrition and malabsorption due to exocrine insufficiency
Psychological sequelae.
Prevention Strategies:
Long-term management of underlying hypertriglyceridemia is crucial
This includes lifestyle modifications (dietary changes, weight management, regular exercise), and pharmacotherapy (fibrates, statins, niacin, omega-3 fatty acids) as guided by a lipid specialist
Regular follow-up with lipid profile monitoring
Genetic counseling for families with inherited dyslipidemias
Patient and family education on the condition, triggers, and adherence to treatment.
Prognosis
Factors Affecting Prognosis:
Severity of pancreatitis (e.g., presence of necrosis, organ failure), promptness of diagnosis and initiation of treatment, triglyceride levels at presentation, presence of underlying genetic disorders, and development of complications
The Ranson score or BISAP score can be used to assess severity in adults, but age-specific scoring systems are less established in pediatrics.
Outcomes:
With prompt and aggressive management, many children and adolescents can recover from acute HTGP without long-term sequelae
However, severe cases can lead to significant morbidity and mortality
Recurrence is possible if hypertriglyceridemia is not adequately controlled
Long-term management of dyslipidemia is key to preventing future episodes.
Follow Up:
Regular follow-up with pediatric gastroenterologists and endocrinologists is essential
This includes periodic lipid profile monitoring, adherence assessment for lifestyle modifications and medications, and monitoring for signs of chronic pancreatitis or endocrine dysfunction (e.g., diabetes)
Educational sessions for patients and families on disease management and prevention of recurrence are vital.
Key Points
Exam Focus:
HTGP is a significant cause of pancreatitis in adolescents with triglyceride levels >1000 mg/dL
Prompt triglyceride reduction (insulin infusion, plasmapheresis) is key
Differentiate from other causes of pediatric pancreatitis
Recognize signs of severe disease and complications.
Clinical Pearls:
Always consider hypertriglyceridemia in a child or adolescent with acute pancreatitis, especially if obese or with a family history of dyslipidemia
Do not delay insulin therapy even in the absence of hyperglycemia
Aggressive fluid resuscitation is crucial
Long-term lipid management is as important as acute management.
Common Mistakes:
Underestimating the severity of HTGP
Delaying aggressive triglyceride-lowering strategies
Over-reliance on imaging without prompt laboratory assessment of triglycerides
Inadequate pain control
Insufficient nutritional support or inappropriate route of feeding
Failing to address the underlying cause of hypertriglyceridemia for long-term prevention.