Define Portal Hypertension in Children. Describe its classification, clinical features, investigations and management.

Definition

• Portal hypertension is a sustained increase in the pressure within the portal venous system (portal pressure > 10–12 mmHg, or a hepatic venous pressure gradient > 5 mmHg).
• It leads to the opening of porto-systemic collaterals, splenomegaly and the risk of variceal bleeding.

Classification BY Site of Obstruction

Clinical Features

• Splenomegaly (often massive) — a common presenting sign; may cause hypersplenism (anaemia, leucopenia, thrombocytopenia).
• Upper GI bleeding from oesophageal/gastric varices — haematemesis and/or melena (often the first and dramatic presentation).
• Ascites and dilated abdominal wall veins — more with intrahepatic (cirrhotic) causes.
• Features of the underlying liver disease (jaundice, growth failure) in intrahepatic causes.

Investigations

• Ultrasound with Doppler — assesses the portal vein (thrombosis/cavernoma in EHPVO), liver echotexture, spleen size and collaterals.
• Upper GI endoscopy — detects and grades oesophageal/gastric varices.
• Liver function tests, CBC (hypersplenism), coagulation profile; liver biopsy if intrinsic liver disease is suspected.

Management — Acute Variceal Bleed

• Resuscitation first — airway, breathing, IV access, restore circulation (cautious transfusion), correct coagulopathy.
• Vasoactive drugs — octreotide/somatostatin (or terlipressin) to reduce portal pressure.
• Endoscopic therapy — variceal band ligation (preferred) or sclerotherapy.
• Balloon tamponade as a temporary bridge; prophylactic antibiotics.

Management — Long-term

• Non-selective beta-blockers (propranolol) and endoscopic obliteration to prevent re-bleeding.
• Shunt surgery / Meso-Rex bypass (especially in EHPVO) for uncontrolled bleeding.
• Treat the underlying cause; manage hypersplenism and ascites; liver transplant for end-stage cirrhosis.

Pathophysiology

Obstruction to portal flow (pre-/intra-/post-hepatic) → Rise in portal venous pressure → Opening of porto-systemic collaterals + splenomegaly → Varices (oesophageal/gastric) → GI bleeding; ascites (intrahepatic)

Sites of Porto-systemic Collaterals

• Oesophageal & gastric varices (the ones that bleed dangerously).
• Rectal (haemorrhoids), umbilical (caput medusae), and retroperitoneal collaterals.

Extrahepatic Portal Vein Obstruction (ehpvo)

• The commonest cause in Indian children; the obstructed portal vein is replaced by a network of collaterals (a 'portal cavernoma').
• Risk factors — neonatal umbilical sepsis or umbilical vein catheterisation, dehydration, prothrombotic states.
• Liver function is preserved, so ascites and encephalopathy are uncommon; growth retardation can occur.

Complications

• Recurrent variceal bleeding; hypersplenism (cytopenias); ascites (intrahepatic causes).
• Hepatic encephalopathy and portal biliopathy (in EHPVO); growth failure.

Prognosis

• EHPVO has a good long-term prognosis because liver function is preserved — the main challenge is controlling variceal bleeding, which can often be managed endoscopically or by a shunt (Meso-Rex).
• Prognosis in intrahepatic causes depends on the underlying liver disease.

Primary Prophylaxis of Bleeding

• Screening endoscopy detects varices at risk of bleeding.
• Non-selective beta-blockers (propranolol) and/or prophylactic band ligation reduce the risk of a first bleed.

Management of Other Features

• Hypersplenism — usually needs no specific treatment unless severe cytopenias occur.
• Ascites (intrahepatic causes) — salt restriction and diuretics.
• Nutritional support and growth monitoring, especially in EHPVO.