Portal Hypertensive Enteropathy

Mucosal and vascular changes in the small bowel (duodenum, jejunum, ileum) in portal hypertension: erythema, villous oedema, vascular ectasia (angioectasia), petechiae, and small bowel varices.

• Found by capsule endoscopy in about 40 to 70% of patients with cirrhosis (more with advanced disease and large oesophageal varices); duodenal involvement is common; most patients are asymptomatic.
• Accounts for about 5 to 10% of obscure gastrointestinal bleeding in portal hypertension and contributes to iron deficiency anaemia and protein losing enteropathy.
• Causes: cirrhosis, schistosomiasis, extrahepatic portal vein obstruction, Budd-Chiari syndrome, and non-cirrhotic portal hypertension.
• Mechanism: raised mesenteric venous pressure causes congestion, capillary dilatation and ectasia, mucosal oedema, increased permeability, and altered motility and bacterial overgrowth.

Often silent; occult bleeding with chronic iron deficiency anaemia, melaena or maroon stools, fatigue; diarrhoea and weight loss from malabsorption or protein loss; hypoalbuminaemia and oedema in protein losing enteropathy.

• Features of portal hypertension: ascites, splenomegaly, caput medusae, jaundice, previous variceal bleeding.
• Differential diagnosis: NSAID enteropathy, angioectasia unrelated to portal hypertension, Crohn disease, coeliac disease, small bowel tumours, Meckel diverticulum, hookworm and other infection, portal hypertensive gastropathy and colopathy, ectopic varices.
• Complications: chronic anaemia, obscure gastrointestinal bleeding with transfusion dependence, protein loss, bacterial overgrowth, ectopic variceal haemorrhage, and encephalopathy precipitated by bleeding.

Capsule endoscopy (the preferred test for obscure bleeding after normal upper and lower endoscopy): red spots, cherry-red erythema, angioectasia, villous oedema ("snowflake" or "mosaic" pattern), and varices; check that the capsule can pass (patency capsule if stricture is suspected).

• Device-assisted (balloon or spiral) enteroscopy: confirms lesions and allows treatment; CT or MR enterography and CT angiography for varices and bleeding source; mesenteric angiography for active bleeding.
• Blood tests: full blood count, ferritin, iron profile, albumin, INR, bilirubin, creatinine, coeliac serology (tissue transglutaminase IgA), faecal calprotectin; alpha-1 antitrypsin clearance for protein loss; stool for ova and parasites.
• Upper endoscopy and colonoscopy to exclude other sources and to document gastric and colonic changes; Doppler ultrasound for portal vein patency.

Correct anaemia and nutrition, reduce portal pressure, and treat bleeding endoscopically or by radiological shunting.

• Iron replacement: ferrous sulfate 200 mg once daily or on alternate days for 3 to 6 months, or ferric carboxymaltose 1000 mg IV once (maximum 20 mg/kg) when oral iron fails; transfuse for haemoglobin under 70 g/L (80 g/L in cardiovascular disease) or symptoms.
• Reduce portal pressure: carvedilol 6.25 mg once daily increasing to 6.25 mg twice daily, or propranolol 20 to 40 mg twice daily titrated to heart rate 55 to 60 beats/min (maximum 160 mg daily without ascites, 80 mg with ascites); stop with refractory ascites and systolic pressure under 90 mmHg (evidence in enteropathy is limited).
• Acute bleeding:
  • Resuscitation, restrictive transfusion (haemoglobin 70 to 80 g/L), correction of severe coagulopathy, high-dependency care.
  • Terlipressin 2 mg IV every 4 hours for 48 hours then 1 mg every 4 hours, or octreotide 50 micrograms IV bolus then 50 micrograms/hour for 2 to 5 days; ceftriaxone 1 g IV daily for up to 7 days; lactulose 30 mL every 8 hours.
  • Endoscopic therapy at enteroscopy: argon plasma coagulation (30 to 40 watts, 0.8 to 1 L/min), haemoclips, or band ligation of ectopic varices; cyanoacrylate injection for larger varices.
  • Refractory bleeding: angiographic embolisation, transjugular intrahepatic portosystemic shunt with embolisation of varices, surgery, or liver transplantation.
• Recurrent bleeding from angioectasia: octreotide long-acting release 20 to 30 mg IM every 4 weeks (evidence limited); avoid tranexamic acid in cirrhosis because of thrombosis risk unless bleeding is life threatening.
• Nutrition and protein loss: high-protein diet (1.2 to 1.5 g/kg/day) with a late evening snack, albumin infusion only for specific indications, treatment of bacterial overgrowth with rifaximin 550 mg twice daily for 2 weeks (or 400 mg three times daily for 7 to 10 days).
• Treat the cause: 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 once), hookworm (albendazole 400 mg once), portal vein thrombosis (anticoagulation under specialist care), and liver transplantation when decompensated.
• Avoid NSAIDs and aspirin unless essential.
• Special groups: children (extrahepatic portal vein obstruction; iron 3 to 6 mg/kg/day of elemental iron, propranolol 1 mg/kg/day, shunt surgery); pregnancy (iron, octreotide for bleeding, avoid terlipressin, capsule endoscopy only when essential); elderly (screen for cancer and angioectasia unrelated to portal hypertension); renal impairment (avoid NSAIDs and aminoglycosides; adjust IV iron and octreotide); anticoagulated patients (review need).
• Red flags: melaena or haematochezia with shock, haemoglobin fall of 20 g/L or more, rebleeding despite endoscopic therapy, jaundice, ascites with fever, encephalopathy, rapid weight loss, severe hypoalbuminaemia.
• Follow-up: haemoglobin and ferritin at 4 to 8 weeks and every 3 months; repeat capsule endoscopy or enteroscopy for recurrent bleeding; liver tests and portal hypertension review every 3 months; liver ultrasound every 6 months; vaccination (hepatitis A and B, influenza, pneumococcal).