Chronic liver disease, cirrhosis and portal hypertension: SAQ

This boy with known biliary atresia presents the classic picture of acute variceal bleeding, the most feared complication of portal hypertension in children. The history of biliary atresia with Kasai portoenterostomy marks him as a child at high risk for progressive fibrosis and portal hypertension. The massive haematemesis with tachycardia, cool peripheries, and a haemoglobin of 72 g per litre indicates significant acute blood loss with early hypovolaemic shock. The splenomegaly, thrombocytopenia of 85,000 per cubic millimetre, and INR of 1.8 confirm long-standing portal hypertension with hypersplenism and impaired synthetic function. ^[1]

Question 1 (10 marks)

Outline your immediate management of this child in the emergency department, including resuscitation, pharmacological therapy, and endoscopic intervention. ^[1]

Management begins with airway, breathing, and circulation. The airway is assessed and protected, as massive haematemesis carries a risk of aspiration, particularly in a child who may become encephalopathic. Two large-bore intravenous cannulae are inserted and crystalloid resuscitation is initiated. Blood is cross-matched and transfused to a haemoglobin target of approximately 7 to 8 g per decilitre. Overtransfusion must be avoided because it raises portal pressure and may worsen bleeding, so the target is deliberately below the normal range. ^[1]

Vasoactive therapy should be started immediately. Octreotine is given as a 1 to 2 micrograms per kg bolus followed by a continuous infusion of 1 to 5 micrograms per kg per hour. This reduces splanchnic blood flow and portal pressure, helping to control the active bleed and reduce early rebleeding. Terlipressin at 0.04 to 0.08 mg per kg every 4 to 6 hours is an alternative supported by adult meta-analysis evidence showing a survival benefit for acute variceal bleeding. A meta-analysis of randomised controlled trials confirmed that terlipressin is superior to placebo for achieving initial haemostasis and improving survival in acute variceal bleeding. ^[3]

Prophylactic antibiotics are mandatory. A third-generation cephalosporin such as ceftriaxone at 50 to 100 mg per kg per day is given for 5 to 7 days, as bacterial infection including spontaneous bacterial peritonitis is common in cirrhotic patients with acute bleeding and antibiotic prophylaxis improves survival. The child's coagulopathy should be addressed with vitamin K, and fresh frozen plasma or platelets may be considered if there is active bleeding with severe coagulopathy, though the evidence for routine correction is limited. ^[2]

Once haemodynamically stabilised, the child undergoes urgent upper gastrointestinal endoscopy, ideally within 12 to 24 hours. Endoscopic variceal band ligation is the first-line endoscopic therapy for oesophageal variceal bleeding. If bleeding cannot be controlled, balloon tamponade with a Sengstaken-Blakemore tube provides temporary haemostasis as a bridge to definitive therapy such as a transjugular intrahepatic portosystemic shunt. The paediatric hepatology, anaesthetic, and surgical teams should be involved early. ^[1]