PE23.16 | Portal Hypertension — Summary & Reflection

KEY TAKEAWAYS

Portal hypertension in children is classified as pre-hepatic (EHPVO — commonest in India, normal liver function), intrahepatic (cirrhotic, from any CLD cause), or post-hepatic (Budd-Chiari syndrome). EHPVO presents with massive splenomegaly and variceal haemorrhage with completely normal LFTs and a Doppler USG showing cavernous transformation of the portal vein. The pathophysiology is elevated portal venous pressure from obstruction, leading to portosystemic collateral (variceal) formation, splenomegaly, and hypersplenism. Acute variceal haemorrhage management: IV resuscitation (PRBC to Hb 7–8, avoid over-transfusion) + IV octreotide + IV ceftriaxone + urgent OGD with band ligation within 12–24 hours. The Sengstaken-Blakemore tube is a bridge for uncontrolled bleeding. Chronic management: EVL programme (secondary prophylaxis) + propranolol. For EHPVO with patent intrahepatic left portal vein: the Rex shunt (meso-Rex bypass) is the preferred definitive surgical option, restoring physiological portal flow. Cirrhotic PHT additionally requires ascites management (spironolactone/furosemide), SBP prophylaxis, and liver transplant evaluation.

REFLECT

A child with massive splenomegaly is brought to your outpatient clinic in a district setting. The spleen reaches the right iliac fossa. The child looks well, eats normally, has no jaundice, and has never vomited blood. LFTs are normal. What would you do next — reassure the family, investigate further, or refer? Consider: what is the risk of a first variceal bleed in this child without treatment? What investigations would you request, and what would you tell the family about the prognosis of EHPVO? Apply Kolb's reflective cycle: describe the clinical situation, analyse what you would do with available resources, conclude by identifying what needs specialist referral, and plan to practise interpreting portal Doppler USG reports on your next rotation.