Overview
Definition:
Upper gastrointestinal (GI) bleeding from esophageal or gastric varices is a life-threatening complication of portal hypertension, commonly seen in pediatric patients with chronic liver disease or congenital portosystemic shunts
Octreotide, a somatostatin analog, is a crucial pharmacologic agent used to reduce splanchnic blood flow and variceal pressure, thereby controlling acute bleeding and preventing rebleeding.
Epidemiology:
The incidence of variceal bleeding in children varies significantly with the underlying etiology of portal hypertension and the severity of liver disease
Children with biliary atresia, idiopathic portal hypertension, and viral hepatitis are at higher risk
Approximately 20-30% of children with documented varices will experience at least one bleeding episode
Mortality rates for variceal bleeds can range from 10-50% depending on the severity and promptness of management.
Clinical Significance:
Variceal bleeding in children is a medical emergency requiring rapid diagnosis and aggressive management
Uncontrolled bleeding can lead to hypovolemic shock, multi-organ failure, and death
Effective management strategies, including pharmacologic therapy with agents like octreotide, are vital for patient survival and improving long-term outcomes, making this a critical topic for pediatric residents preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Hematemesis: Bright red blood or coffee-ground vomitus
Melena: Black, tarry stools
Hematochezia: Less common, but can occur with massive bleeds
Abdominal pain: May be present but often overshadowed by bleeding
Symptoms of underlying liver disease: Jaundice, ascites, hepatosplenomegaly
Symptoms of anemia: Pallor, fatigue, lethargy, dizziness.
Signs:
Hypotension and tachycardia: Indicative of significant blood loss and hypovolemic shock
Pallor: Of conjunctiva and skin
Abdominal distension: Due to ascites or hepatosplenomegaly
Signs of hepatic encephalopathy: Altered mental status, asterixis
Cool, clammy skin
Decreased urine output.
Diagnostic Criteria:
Diagnosis is primarily based on clinical suspicion of upper GI bleeding in a child with known or suspected portal hypertension, confirmed by upper GI endoscopy
Guidelines from organizations like the European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) emphasize prompt endoscopic evaluation
The presence of varices confirmed on endoscopy, coupled with evidence of acute bleeding (active oozing, fresh blood), establishes the diagnosis.
Diagnostic Approach
History Taking:
Detailed history of liver disease (etiology, duration, prior treatments, complications)
History of previous bleeding episodes
Medications (NSAIDs, anticoagulants)
Recent trauma or illness
Family history of liver disease
Assessment of vital signs and hemodynamic stability
Evaluate for signs of dehydration and shock.
Physical Examination:
Systematic assessment of airway, breathing, and circulation
Full abdominal examination to assess for hepatosplenomegaly, ascites, and tenderness
Neurological assessment for hepatic encephalopathy
Examination of skin for pallor, jaundice, and spider angiomata
Rectal examination to assess for melena or hematochezia.
Investigations:
Complete blood count (CBC) with platelets: To assess hemoglobin, hematocrit, and platelet count
Coagulation profile (PT, INR, PTT): To assess synthetic function of the liver and coagulopathy
Liver function tests (LFTs): Bilirubin, albumin, AST, ALT, alkaline phosphatase
Electrolytes, BUN, creatinine: To assess hydration status and renal function
Blood type and crossmatch: For potential blood transfusion
Abdominal ultrasound: To assess liver parenchyma, size of spleen, presence of ascites, and portal vein diameter/flow
Upper GI endoscopy (Esophagogastroduodenoscopy - EGD): The gold standard for diagnosing varices and identifying the source of bleeding
Endoscopic findings include varix size (small, medium, large), presence of red wale marks, and signs of active bleeding.
Differential Diagnosis:
Other causes of upper GI bleeding in children: Esophagitis, gastritis, peptic ulcers, duodenitis, Mallory-Weiss tears, foreign body ingestion, arteriovenous malformations
Differentiating variceal bleeding is crucial due to its unique management strategy and higher risk of rebleeding.
Management
Initial Management:
Airway, breathing, and circulation (ABC) assessment
Establish intravenous access (large bore cannulas)
Fluid resuscitation with crystalloids (e.g., Normal Saline, Lactated Ringer's) to maintain hemodynamic stability
Blood transfusion: To maintain hemoglobin >7-8 g/dL (or >9-10 g/dL in active bleeding or significant comorbidities)
Correct coagulopathy with fresh frozen plasma (FFP), vitamin K, or platelet transfusion as indicated.
Medical Management:
Octreotide: Administer an initial bolus of 1-2 mcg/kg intravenously, followed by a continuous infusion of 1-2 mcg/kg/hour
This reduces portal pressure by inhibiting the release of vasodilatory hormones and decreasing splanchnic blood flow
Vasopressin: Historically used, but less favored due to cardiac side effects and lower efficacy compared to octreotide in many settings
Somatostatin: Similar mechanism to octreotide, but shorter half-life
Proton Pump Inhibitors (PPIs): Intravenous PPIs (e.g., pantoprazole, omeprazole) are essential to reduce gastric acidity and promote clot stability, given at high doses (e.g., 4 mg/kg/day divided every 12 hours in children, max 40 mg/dose)
Antibiotic prophylaxis: Essential in cirrhotic patients with variceal bleeding to prevent spontaneous bacterial peritonitis (SBP)
Norfloxacin or ciprofloxacin are commonly used orally or intravenously.
Endoscopic Management:
Endoscopic variceal ligation (EVL) or endoscopic sclerotherapy (EST) are the mainstay of definitive management once the acute bleed is controlled
EVL involves placing rubber bands around varices, while EST involves injecting a sclerosant agent into the varices
The choice between EVL and EST depends on varix characteristics and endoscopist expertise
Combined pharmacologic and endoscopic therapy offers the best chance of controlling acute bleeding.
Surgical Management:
Surgical interventions are typically reserved for refractory bleeding that does not respond to medical and endoscopic therapies, or for secondary prophylaxis
Options include: Transjugular Intrahepatic Portosystemic Shunt (TIPS): Creates a tract between the portal vein and hepatic vein to decompress portal pressure
Pediatric use is less common and requires specialized centers
Surgical shunts (e.g., mesocaval, splenorenal): Rarely performed in the current era due to their morbidity
Distal splenorenal shunt (Warren shunt) is the most commonly considered surgical option
Esophageal transection or devascularization: Less common, palliative procedures for bleeding control.
Supportive Care:
Continuous hemodynamic monitoring (heart rate, blood pressure, urine output)
Monitor for signs of rebleeding
Nutritional support: Maintain adequate nutrition, often requiring nasogastric feeding or parenteral nutrition in severe cases
Monitor for electrolyte imbalances and fluid overload, especially in cirrhotic patients
Close monitoring for complications such as hepatic encephalopathy, SBP, and renal dysfunction.
Complications
Early Complications:
Rebleeding: The most common and serious early complication, occurring in 30-50% of patients within the first few weeks
Hypovolemic shock
Organ dysfunction (renal failure, acute respiratory distress syndrome)
Hyponatremia and fluid overload in cirrhotic patients
Sepsis.
Late Complications:
Hepatic encephalopathy: Worsening or new onset
Ascites: Recurrent or refractory
Development of hepatocellular carcinoma (HCC) in patients with underlying chronic liver disease
Progressive liver failure
Nutritional deficiencies.
Prevention Strategies:
Adherence to prescribed prophylactic pharmacotherapy (octreotide/vasopressin/somatostatin, beta-blockers for secondary prophylaxis)
Regular endoscopic surveillance for varices and their status
Early and aggressive management of acute bleeding
Prompt initiation of antibiotic prophylaxis in cirrhotic patients
Close monitoring and management of underlying liver disease.
Prognosis
Factors Affecting Prognosis:
Severity of underlying liver disease (Child-Pugh score)
Presence and severity of complications (encephalopathy, ascites, renal dysfunction)
Promptness and efficacy of initial management
Etiology of portal hypertension
Number and size of varices
Red wale marks and presence of active bleeding at endoscopy
Recurrent bleeding episodes.
Outcomes:
With prompt and appropriate management, the immediate mortality from a single variceal bleeding episode can be reduced significantly
However, the long-term prognosis remains guarded, heavily dependent on the progression of the underlying liver disease
Patients with well-compensated liver function have better outcomes
Recurrent bleeding is a major determinant of long-term survival.
Follow Up:
Patients who have experienced variceal bleeding require lifelong follow-up
This includes regular clinical assessment, serial LFTs, and periodic endoscopic surveillance (typically every 6-12 months, or more frequently if indicated) to monitor for varix recurrence or development of new varices
Management of portal hypertension with non-selective beta-blockers (e.g., propranolol, nadolol) is often initiated for secondary prophylaxis once the acute bleed is controlled, although their use in pediatrics is less standardized than in adults and requires careful consideration of side effects and efficacy
Liver transplantation may be the definitive treatment for end-stage liver disease.
Key Points
Exam Focus:
Octreotide mechanism of action: Decreases splanchnic blood flow and portal pressure
Dosing for octreotide in pediatric variceal bleed: Bolus followed by infusion
Role of PPIs and antibiotics in variceal bleeding management
Endoscopic management: EVL vs
EST
Indications for TIPS and surgical shunts
Risk factors for rebleeding.
Clinical Pearls:
Always consider octreotide in any child with acute upper GI bleed and known or suspected portal hypertension
Early endoscopic evaluation is paramount for diagnosis and definitive management
Do not underestimate the risk of rebleeding
vigilant monitoring is key
Antibiotic prophylaxis is non-negotiable in cirrhotic patients with variceal bleeding to prevent SBP.
Common Mistakes:
Delayed initiation of octreotide infusion, leading to continued blood loss
Failure to provide adequate fluid resuscitation and blood products
Not administering PPIs concurrently with octreotide
Inadequate antibiotic prophylaxis
Underestimating the need for intensive monitoring post-endoscopy and post-treatment.