Esophageal Varices in Children

Esophageal Varices Introduction Etiology Pathophysiology Clinical Presentation Diagnosis Management Complications Prognosis

Introduction to Esophageal Varices in Children

Esophageal varices are abnormally dilated submucosal veins in the lower esophagus, typically resulting from portal hypertension. While more common in adults, they can occur in children and represent a significant cause of upper gastrointestinal bleeding in pediatric patients with liver disease or portal vein thrombosis.

Key points:

• Prevalence: Approximately 50-80% of children with cirrhosis develop esophageal varices
• Age: Can occur at any age, but more common in older children and adolescents with chronic liver disease
• Mortality: Variceal bleeding carries a mortality rate of 5-19% in children

Etiology of Esophageal Varices in Children

The primary cause of esophageal varices in children is portal hypertension, which can result from various conditions:

1. Intrahepatic causes:
   • Cirrhosis (e.g., biliary atresia, autoimmune hepatitis, Wilson's disease)
   • Congenital hepatic fibrosis
   • Cystic fibrosis-associated liver disease
2. Extrahepatic causes:
   • Portal vein thrombosis
   • Budd-Chiari syndrome
   • Congenital portosystemic shunts
3. Idiopathic portal hypertension

Understanding the underlying etiology is crucial for appropriate management and long-term prognosis.

Pathophysiology of Esophageal Varices in Children

The development of esophageal varices involves complex hemodynamic changes:

1. Portal hypertension: Increased resistance to portal blood flow leads to elevated portal pressure (normal: 5-10 mmHg; portal hypertension: >10 mmHg).
2. Collateral circulation: To decompress the portal system, collateral vessels form, including those in the distal esophagus and proximal stomach.
3. Variceal formation: These collateral vessels dilate over time, forming varices.
4. Increased wall tension: As varices enlarge, wall tension increases, raising the risk of rupture and bleeding.

Factors contributing to variceal bleeding:

• Elevated portal pressure (>12 mmHg)
• Large varix size
• Thin varix wall
• Endothelial injury (e.g., from reflux esophagitis)

Clinical Presentation of Esophageal Varices in Children

Esophageal varices may be asymptomatic until bleeding occurs. Presentation can include:

• Acute variceal bleeding:
  • Hematemesis (vomiting of blood)
  • Melena (black, tarry stools)
  • Hematochezia (bright red blood per rectum) in severe cases
  • Signs of hypovolemic shock (tachycardia, hypotension, pallor)
• Chronic liver disease signs:
  • Jaundice
  • Ascites
  • Spider angiomas
  • Palmar erythema
• Portal hypertension signs:
  • Splenomegaly
  • Caput medusae (dilated periumbilical veins)

Note: The severity of presentation can vary widely, from incidental findings to life-threatening hemorrhage.

Diagnosis of Esophageal Varices in Children

Diagnostic approach includes:

1. History and physical examination
2. Laboratory tests:
   • Complete blood count
   • Liver function tests
   • Coagulation profile
   • Blood type and cross-match
3. Imaging studies:
   • Abdominal ultrasound with Doppler: Evaluate liver, spleen, and portal venous system
   • CT or MRI: Assess liver parenchyma and vascular anatomy
4. Endoscopy:
   • Gold standard for diagnosis and grading of varices
   • Grading system:
     • Grade I: Small, straight varices
     • Grade II: Enlarged, tortuous varices occupying <1/3 of the esophageal lumen
     • Grade III: Large varices occupying >1/3 of the esophageal lumen
   • Red wale signs: Indicate high risk of bleeding
5. Hepatic venous pressure gradient (HVPG) measurement:
   • Invasive procedure, not routinely performed in children
   • HVPG >10 mmHg indicates clinically significant portal hypertension

Management of Esophageal Varices in Children

Management strategies include prevention, acute bleeding control, and long-term care:

1. Primary Prophylaxis

• Non-selective beta-blockers (e.g., propranolol): Reduce portal pressure
• Endoscopic variceal ligation (EVL): For large varices at high risk of bleeding

2. Acute Variceal Bleeding Management

• Resuscitation:
  • Fluid resuscitation
  • Blood product transfusion (target Hb: 7-9 g/dL)
• Pharmacological therapy:
  • Octreotide: Reduces splanchnic blood flow
  • Proton pump inhibitors
  • Antibiotics (e.g., ceftriaxone): Prevent bacterial infections
• Endoscopic therapy:
  • EVL: Preferred method
  • Sclerotherapy: Alternative if EVL not feasible
• Balloon tamponade (e.g., Sengstaken-Blakemore tube): For refractory bleeding

3. Secondary Prophylaxis

• Combination of non-selective beta-blockers and EVL
• Regular endoscopic surveillance

4. Advanced Interventions

• Transjugular intrahepatic portosystemic shunt (TIPS): For refractory bleeding
• Surgical shunts: Reserved for specific cases (e.g., extrahepatic portal vein obstruction)
• Liver transplantation: Definitive treatment for end-stage liver disease

Complications of Esophageal Varices in Children

Major complications include:

1. Variceal hemorrhage:
   • Life-threatening emergency
   • Risk of hypovolemic shock and death
2. Hepatic encephalopathy:
   • Due to blood loss and increased protein load in the GI tract
   • Can be precipitated by variceal bleeding
3. Aspiration pneumonia:
   • Risk during active bleeding
4. Infection:
   • Spontaneous bacterial peritonitis
   • Bacteremia
5. Esophageal strictures:
   • Can occur after repeated endoscopic treatments
6. Portal vein thrombosis:
   • Risk increased in children with cirrhosis and varices

Prognosis of Esophageal Varices in Children

The prognosis for children with esophageal varices depends on several factors:

• Underlying cause of portal hypertension
• Severity of liver disease
• Presence of other complications
• Timely access to appropriate medical care

Key prognostic considerations:

1. Mortality:
   • 6-week mortality after variceal bleeding: 5-19%
   • Lower than in adults due to better liver reserve in children
2. Rebleeding risk:
   • 30-40% within 6 weeks of initial bleeding episode
   • Reduced with proper secondary prophylaxis
3. Long-term outlook:
   • Improved with advances in endoscopic and pharmacological management
   • Liver transplantation offers good outcomes for end-stage liver disease
4. Quality of life:
   • Can be significantly impacted by repeated hospitalizations and procedures
   • Psychosocial support is crucial for patients and families

Regular follow-up and adherence to management plans are essential for optimizing outcomes in children with esophageal varices.

Esophageal Varices in Children

• Q1: What are esophageal varices?
  A: Dilated blood vessels in the lower esophagus that can rupture and bleed.
• Q2: What is the most common cause of esophageal varices in children?
  A: Portal hypertension, often due to chronic liver disease.
• Q3: Which imaging technique is most commonly used to diagnose esophageal varices in children?
  A: Upper endoscopy (esophagogastroduodenoscopy or EGD).
• Q4: What is the most life-threatening complication of esophageal varices?
  A: Massive gastrointestinal bleeding.
• Q5: What are the signs of acute bleeding from esophageal varices?
  A: Hematemesis (vomiting blood), melena (black, tarry stools), and signs of shock.
• Q6: Which medication class is commonly used for primary prophylaxis of variceal bleeding?
  A: Non-selective beta-blockers (e.g., propranolol).
• Q7: What is the role of endoscopic variceal ligation (EVL) in managing esophageal varices?
  A: It's used for both primary and secondary prophylaxis of variceal bleeding.
• Q8: What is the name of the surgical procedure that can be used to treat portal hypertension and reduce the risk of variceal bleeding?
  A: Portosystemic shunt surgery (e.g., transjugular intrahepatic portosystemic shunt or TIPS).
• Q9: Which laboratory test is essential in assessing the severity of liver disease in children with esophageal varices?
  A: Prothrombin time (PT) or International Normalized Ratio (INR).
• Q10: What is the recommended initial fluid resuscitation for a child with acute variceal bleeding?
  A: Isotonic crystalloid solution (e.g., normal saline).
• Q11: Which vitamin deficiency is common in children with chronic liver disease and esophageal varices?
  A: Vitamin K deficiency.
• Q12: What is the term for the presence of both esophageal and gastric varices?
  A: Gastroesophageal varices.
• Q13: Which scoring system is used to grade the severity of esophageal varices?
  A: The Paquet grading system (Grades I-IV).
• Q14: What is the role of octreotide in managing acute variceal bleeding?
  A: It reduces splanchnic blood flow and portal pressure, helping to control bleeding.
• Q15: How often should surveillance endoscopies be performed in children with known esophageal varices?
  A: Usually every 6-12 months, depending on the severity and underlying condition.
• Q16: What is the most appropriate initial action when a child presents with suspected variceal bleeding?
  A: Assess and stabilize airway, breathing, and circulation (ABCs).
• Q17: Which non-invasive test can be used to assess the degree of liver fibrosis in children with portal hypertension?
  A: Transient elastography (FibroScan).
• Q18: What is the name of the phenomenon where blood flow in the portal vein reverses direction?
  A: Hepatofugal flow.
• Q19: Which antibiotic is commonly used for prophylaxis in children with variceal bleeding?
  A: Ceftriaxone.
• Q20: What is the recommended timing for initiating oral feeding after an episode of variceal bleeding?
  A: Usually 24-48 hours after bleeding has stopped, if the patient is stable.
• Q21: What is the term for the development of new blood vessels between the portal and systemic circulations in portal hypertension?
  A: Portosystemic collaterals.
• Q22: Which imaging modality can be used to assess portal vein patency and flow direction?
  A: Doppler ultrasound.
• Q23: What is the primary goal of beta-blocker therapy in children with esophageal varices?
  A: To reduce portal pressure and prevent variceal bleeding.
• Q24: Which liver disease is the most common cause of portal hypertension and esophageal varices in children in developed countries?
  A: Biliary atresia.
• Q25: What is the term for the presence of varices in the stomach?
  A: Gastric varices.
• Q26: Which blood product is often given to correct coagulopathy in children with liver disease and variceal bleeding?
  A: Fresh frozen plasma (FFP).
• Q27: What is the name of the procedure where a balloon is inserted into the esophagus to compress bleeding varices?
  A: Balloon tamponade (e.g., Sengstaken-Blakemore tube).
• Q28: Which radiological procedure can be used to embolize bleeding varices?
  A: Transjugular intrahepatic portosystemic shunt (TIPS) with embolization.
• Q29: What is the term for the accumulation of fluid in the abdominal cavity often seen in children with portal hypertension?
  A: Ascites.
• Q30: Which endoscopic technique involves injecting a sclerosing agent directly into the varices?
  A: Endoscopic sclerotherapy.

Further Reading

• Management of Variceal Bleeding in Children - Comprehensive review article from the World Journal of Hepatology
• NASPGHAN Practice Guidelines: Diagnosis and Management of Portal Hypertension in Children - Clinical practice guidelines from the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition
• Prevention and Management of Gastroesophageal Varices in Children - Evidence-based recommendations from the Journal of Pediatric Gastroenterology and Nutrition
• Portal hypertension and its complications in children - In-depth review from The Lancet Gastroenterology & Hepatology