Oesophageal Varices

Dilated submucosal veins of the lower oesophagus that form portosystemic collaterals when portal pressure rises; they bleed when the wall tension exceeds its strength.

• Causes: cirrhosis (alcohol, hepatitis B and C, metabolic dysfunction related steatotic liver disease), schistosomiasis (periportal fibrosis; a major cause in Africa), portal vein thrombosis, Budd-Chiari syndrome, idiopathic non-cirrhotic portal hypertension, and congenital hepatic fibrosis.
• Varices form when the hepatic venous pressure gradient is 10 mmHg or more and bleed at 12 mmHg or more; about 30 to 40% of compensated and 60% of decompensated cirrhosis patients have varices at diagnosis, and small varices progress to large at about 5 to 10% per year.
• Bleeding occurs in about 5 to 15% a year (15 to 20% with large varices); each episode carries about 15 to 20% mortality at 6 weeks, and rebleeding without prevention is about 60% at 1 to 2 years.
• Mechanism: raised resistance in the liver, with splanchnic vasodilatation and increased inflow.

Asymptomatic until bleeding occurs; haematemesis (large volume of fresh blood), melaena, syncope, shock, and often a background of chronic liver disease.

• Stigmata of liver disease: jaundice, ascites, spider naevi, palmar erythema, gynaecomastia, splenomegaly, caput medusae, encephalopathy, muscle wasting.
• Examination in a bleed: tachycardia, hypotension, pallor, confusion.
• Differential diagnosis: peptic ulcer, erosive gastritis, Mallory-Weiss tear, portal hypertensive gastropathy, gastric varices, Dieulafoy lesion, angiodysplasia, oesophagitis, cancer.
• Complications: haemorrhagic shock, aspiration, hepatic encephalopathy, bacterial infection (spontaneous bacterial peritonitis, pneumonia), acute kidney injury and hepatorenal syndrome, liver decompensation, rebleeding, and death.

Upper endoscopy classifies varices by size (small, less than 5 mm; medium or large, more than 5 mm) and by red signs (red wale marks, cherry-red spots) that predict bleeding, and shows gastric varices and portal hypertensive gastropathy.

• Screening: endoscopy at diagnosis of cirrhosis, unless platelets are more than 150 x 10^9/L and liver stiffness by transient elastography is less than 20 kPa (low risk; repeat yearly).
• Surveillance: no varices, repeat endoscopy every 2 years (yearly if decompensated); small varices every 1 year.
• Blood tests: full blood count (low platelets), INR, albumin, bilirubin, creatinine, electrolytes, liver tests, group and crossmatch, blood cultures if febrile; hepatitis serology, HIV test, schistosomiasis serology or stool and urine microscopy.
• Ultrasound with Doppler and CT or MR angiography: liver morphology, portal vein patency, splenomegaly, collaterals, hepatic vein thrombosis, hepatocellular carcinoma.

Prevent the first bleed, treat the acute bleed in a high-dependency setting, and prevent rebleeding and treat the liver disease.

• Primary prophylaxis:
  • Small varices with red signs or in advanced cirrhosis, and all medium or large varices: carvedilol 6.25 mg once daily, increased after 3 days to 6.25 mg twice daily (maximum 12.5 mg daily; stop if systolic pressure is less than 90 mmHg); or propranolol 20 to 40 mg twice daily, titrated to a resting heart rate of 55 to 60 beats/min (maximum 160 mg daily without ascites, 80 mg daily with ascites); or nadolol 20 to 40 mg once daily (maximum 80 mg daily without ascites, 40 mg with).
  • Endoscopic variceal band ligation every 2 to 4 weeks until eradication for medium or large varices when beta blockers are contraindicated or not tolerated, then endoscopy at 3 to 6 months and every 6 to 12 months.
  • Stop beta blockers with refractory ascites and systolic pressure under 90 mmHg, sodium under 130 mmol/L, hepatorenal syndrome or acute kidney injury.
• Acute variceal bleeding (resuscitation first):
  • Airway protection (intubation for encephalopathy or massive haematemesis), two large-bore cannulae, careful volume replacement (restrictive transfusion at haemoglobin of 70 g/L, target 70 to 80 g/L; avoid over-transfusion, which raises portal pressure), correction of coagulopathy only for severe deficiency (platelets under 50 x 10^9/L with active bleeding).
  • Vasoactive drug started as soon as bleeding is suspected and continued for 2 to 5 days: terlipressin 2 mg IV every 4 hours for the first 48 hours then 1 mg every 4 hours (maximum 5 days; avoid in ischaemic heart or peripheral vascular disease, severe hypoxia); or octreotide 50 micrograms IV bolus then 50 micrograms/hour infusion.
  • Antibiotic prophylaxis: ceftriaxone 1 g IV daily for up to 7 days (or until bleeding stops and discharge).
  • Erythromycin 250 mg IV 30 to 120 minutes before endoscopy to clear the stomach (check QT).
  • Endoscopy within 12 hours: band ligation of the bleeding varix (preferred), or sclerotherapy with ethanolamine or cyanocobalamin if ligation is not possible.
  • Treat encephalopathy risk: lactulose 30 mL every 8 hours orally or by nasogastric tube (titrate to 2 to 3 soft stools daily) or rectally; avoid sedatives.
  • Bleeding not controlled: oesophagogastric balloon tamponade tube (maximum 24 hours with the airway secured), self-expanding covered oesophageal metal stent (up to 7 days), and rescue transjugular intrahepatic portosystemic shunt.
  • Pre-emptive transjugular intrahepatic portosystemic shunt within 72 hours (ideally 24 hours) for patients at high risk of failure (advanced liver disease with high bilirubin, low albumin and ascites, or active bleeding at endoscopy despite treatment).
• Secondary prophylaxis (after a bleed): carvedilol 6.25 mg once daily increasing to 6.25 mg twice daily (maximum 12.5 mg daily), or propranolol 20 to 40 mg twice daily titrated to a heart rate of 55 to 60 beats/min, or nadolol 20 to 40 mg once daily, plus band ligation every 2 to 4 weeks until eradication, then endoscopy at 3 to 6 months and then every 6 to 12 months; transjugular intrahepatic portosystemic shunt for rebleeding despite combined therapy; liver transplantation assessment.
• Cause-specific treatment: alcohol abstinence, hepatitis B (tenofovir 245 mg daily or entecavir 0.5 mg daily), hepatitis C (sofosbuvir-velpatasvir 400/100 mg daily for 12 weeks), weight loss in steatotic disease, schistosomiasis (praziquantel 40 mg/kg orally as a single dose, repeated at 4 weeks in heavy infection), portal vein thrombosis (anticoagulation with enoxaparin 1 mg/kg twice daily or rivaroxaban 15 to 20 mg daily for 6 months or longer), Budd-Chiari syndrome (anticoagulation, angioplasty or shunt).
• Special groups: children (propranolol 1 mg/kg/day in 2 to 3 doses, band ligation from about 10 kg, octreotide 1 microgram/kg bolus then 1 microgram/kg/hour, and early transplant referral in biliary atresia); pregnancy (band ligation, propranolol with fetal growth monitoring, octreotide, avoid terlipressin, early delivery planning); elderly (cautious beta blocker titration); renal impairment (avoid NSAIDs and aminoglycosides, albumin 1 g/kg over 2 days for hepatorenal syndrome with terlipressin); hepatocellular carcinoma (oncology plan).
• Red flags: haematemesis, melaena, shock, confusion, fever with ascites, rising creatinine, jaundice, refractory ascites.
• Follow-up: endoscopy every 2 to 4 weeks until eradication, then at 3 to 6 months, then every 6 to 12 months; clinic review every 3 months with liver tests; liver ultrasound every 6 months for hepatocellular carcinoma; vaccination (hepatitis A and B, influenza, pneumococcal).