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]

Question 2 (10 marks)

This child has ascites with shifting dullness on examination. Describe how you would assess for spontaneous bacterial peritonitis, including the diagnostic criterion, and outline the management if it is confirmed. [2]

Spontaneous bacterial peritonitis must be excluded in any child with cirrhotic ascites who is unwell, and this child with acute variceal bleeding is at particularly high risk. A diagnostic paracentesis is performed at the bedside, aspirating ascitic fluid from the left lower quadrant using an aseptic technique. The fluid is sent for cell count and differential, culture in blood culture bottles, albumin, and total protein. [2]

The diagnostic criterion for spontaneous bacterial peritonitis is a polymorphonuclear neutrophil count of 250 or more per cubic millimetre in the ascitic fluid. This threshold is absolute and does not depend on the total cell count, the culture result, or the clinical picture. A PMN count below 250 rules out SBP even if the culture is positive, which is the entity of bacterascites managed differently. The serum-ascites albumin gradient, calculated by subtracting the ascitic albumin from the serum albumin, confirms the ascites is cirrhotic in origin if it is 11 g per litre or more. [2]

If SBP is confirmed, treatment is with a third-generation cephalosporin such as cefotaxime or ceftriaxone at 50 to 100 mg per kg per day for 5 to 7 days, guided by culture and sensitivity results. Intravenous albumin is given at 1.5 g per kg on day 1 and 1 g per kg on day 3 to reduce the risk of hepatorenal syndrome, which complicates up to 30 per cent of SBP episodes. After recovery, secondary prophylaxis with oral norfloxacin or trimethoprim-sulfamethoxazole is continued indefinitely until liver transplantation, as the one-year recurrence rate without prophylaxis is high. SBP is an ominous prognostic marker with a one-year mortality of 50 to 70 per cent and warrants urgent assessment for liver transplantation. [2]