Key points
- In adults the commonest cause is cirrhosis; in children and young adults in India, EHPVO (a blocked portal vein with a normal liver) and non-cirrhotic portal fibrosis are important causes.
- The main danger is variceal bleeding — vomiting blood or passing black stools is an emergency.
- Treatment steps up from beta-blockers and endoscopic banding to TIPS, shunt surgery and, for cirrhosis, liver transplant.
- Young people with a normal liver can do very well with a proximal splenorenal shunt when endoscopic treatment fails.
The causes — and why the liver's condition matters most
| Cause | Who it affects | State of the liver |
|---|---|---|
| Cirrhosis | Adults with alcohol, hepatitis B/C, fatty liver or other chronic liver disease | Scarred; function may be failing |
| Extrahepatic portal vein obstruction (EHPVO) | Children and young adults; often dating from infancy (e.g. umbilical infection, dehydration); sometimes a clotting disorder | Usually normal — the blockage is in the vein outside the liver |
| Non-cirrhotic portal fibrosis (NCPF / idiopathic non-cirrhotic portal hypertension) | Young and middle-aged adults; reported relatively often in India | Liver function preserved; scarring around small portal veins |
| Budd–Chiari syndrome | Adults with blocked hepatic veins, often due to a clotting tendency | Congested; may be damaged |
The distinction is crucial. In cirrhosis, bleeding is often a sign of advanced liver disease and the long-term answer may be a transplant. In EHPVO and NCPF the liver itself works well, so relieving the pressure can give a near-normal life.
How portal hypertension shows itself
- Vomiting blood or black, tarry stools from bleeding varices — see vomiting blood and variceal bleeding.
- A big spleen (splenomegaly) felt as a lump or heaviness on the left; in children it may be found on a routine examination.
- Hypersplenism — the enlarged spleen traps blood cells, causing low platelets, low white cells and anaemia; easy bruising and nosebleeds.
- In cirrhosis: fluid in the abdomen (ascites), jaundice and confusion.
- In children with EHPVO: poor growth, and later, bile duct changes (portal biliopathy).
Variceal bleeding is an emergency
If someone with liver disease, a known big spleen or known varices vomits blood, passes black stools, feels faint or becomes drowsy, go to the nearest hospital with an endoscopy service immediately. Do not wait to see if it stops.
Tests
Upper GI endoscopy grades the varices. Doppler ultrasound and contrast CT show the portal vein, the spleen and any natural shunts, and help plan surgery. Blood tests check liver function and blood counts; FibroScan or a liver biopsy may be needed to separate cirrhosis from NCPF. Young patients with a clotted portal vein are tested for underlying clotting disorders.
The treatment ladder
- 1
Beta-blockers
Carvedilol or propranolol lower portal pressure and reduce the chance of a first or repeat bleed, especially in cirrhosis.
- 2
Endoscopic band ligation (or glue for gastric varices)
Rubber bands placed on oesophageal varices over repeated sessions until they are eradicated. This is the first-line treatment after a bleed for all causes.
- 3
TIPS
A stent placed by an interventional radiologist through the liver, connecting the portal and hepatic veins to lower the pressure. Used mainly in cirrhosis for bleeding that recurs despite banding. It can worsen confusion (encephalopathy) and is not suitable when the portal vein is blocked, as in most EHPVO.
- 4
Shunt surgery or devascularisation
Surgical options for patients with good liver function — particularly EHPVO and NCPF — whose bleeding is not controlled endoscopically, who live far from endoscopy services, or who have a massive spleen with severe hypersplenism.
- 5
Liver transplant
For cirrhosis with portal hypertension and failing liver function. It treats both the pressure and the liver disease. See liver transplant in Kolkata.
Surgical options explained
Proximal splenorenal shunt (PSRS)
The spleen is removed and the splenic vein is joined to the left kidney vein, so high-pressure portal blood drains directly into the body's venous system. It is the operation most commonly used in India for EHPVO and NCPF. It decompresses the varices and corrects hypersplenism in one operation.
Other shunts
Distal splenorenal shunt (spleen preserved) and mesocaval shunts are chosen according to vein anatomy. In children with EHPVO, a Meso-Rex bypass, which restores blood flow into the liver, is considered where the anatomy allows.
Devascularisation (e.g. Sugiura-type or Hassab procedure)
When no suitable vein is available for a shunt, the veins feeding the varices around the stomach and lower oesophagus are divided, usually with removal of the spleen.
Shunt surgery is generally avoided in advanced cirrhosis because it can precipitate liver failure and hepatic encephalopathy, and because it can complicate a later transplant. After removal of the spleen, vaccinations against pneumococcus, meningococcus and Haemophilus are given, and patients are taught to seek prompt care for fever.
What to expect after surgery
Hospital stay after a shunt operation is usually around a week. The shunt is checked by Doppler ultrasound or CT, and a follow-up endoscopy confirms that varices are regressing. For young patients with EHPVO or NCPF and a patent shunt, the risk of further bleeding falls substantially and blood counts improve, allowing normal schooling, work and growth. The main long-term issues are shunt narrowing and, uncommonly, encephalopathy.
Assessment in Kolkata
Dr Bappaditya Har offers portal hypertension surgery and liver transplant assessment at Narayana RN Tagore Hospital, Mukundapur, working with hepatologists, endoscopists and interventional radiologists. Families of young patients who have had repeated bleeds or have a very large spleen can send endoscopy reports, CT scans and blood counts on WhatsApp (+91 70420 39420) for review. Appointments: 080675 06860.
Frequently asked questions
Can portal hypertension be cured?
In EHPVO and NCPF, where the liver is healthy, a successful shunt operation can relieve the problem long-term. In cirrhosis, portal hypertension is controlled with medicines and endoscopy, and only a liver transplant addresses the underlying cause.
What is EHPVO?
Extrahepatic portal vein obstruction is a blockage of the portal vein outside the liver, usually by an old clot. It is a common cause of variceal bleeding and a large spleen in Indian children and young adults, and the liver itself is usually normal.
Should my big spleen be removed?
Removing the spleen alone does not treat portal hypertension and may make things worse. When surgery is needed, splenectomy is usually combined with a shunt or devascularisation.
What is the difference between TIPS and shunt surgery?
TIPS is a stent placed through the liver by a radiologist, used mainly in cirrhosis. Shunt surgery is an open operation, used mainly when the liver is healthy but the portal vein is blocked, as in EHPVO.
How long is recovery after a splenorenal shunt?
Most patients stay in hospital about a week and return to school or work in four to six weeks, with vaccinations and follow-up scans to check the shunt.
Related reading
Vomiting Blood and Black Stools: Variceal Bleeding in Liver Disease →
Vomiting blood or black stools in liver disease can mean bleeding varices, an emergency. What to do, how banding and TIPS work, and transplant review.
Liver Cirrhosis Treatment in Kolkata: Stages, Care and When to Consider a Transplant →
Cirrhosis explained: compensated vs decompensated, Child-Pugh and MELD in plain words, what treatment can and cannot do, and when to consider a transplant.
Liver Transplant in Kolkata →
Evaluation, surgery and recovery — the complete transplant pathway.
Ascites: Fluid in the Abdomen — Causes, Treatment and When It Means Transplant →
Fluid in the abdomen is usually a sign of advanced cirrhosis. Learn the causes, the tap test, salt limits, diuretics, tapping and when transplant helps.
HPB Surgery →
Surgery of the liver, pancreas, gallbladder and bile ducts.
This page is general patient information, not a diagnosis or a substitute for a consultation. Treatment decisions depend on each patient's reports and examination. Read our medical disclaimer and editorial policy.