Portal Hypertension

Introduction

The portal vein is a large vein in the abdomen which carries blood from intestine to the liver. When there is obstruction to its flow, the pressure in the portal vein increases. The blood is forced to flow through alternate channels. The result is swollen veins or varices in the esophagus, stomach, rectum, and umbilical area. These varices rupture and cause internal bleeding which can be life threatening

Types

Portal hypertension can be due to causes in the liver or outside.

• Intrahepatic hypertension: Cirrhosis and hepatic fibrosis scarring are primary intrahepatic causes of portal hypertension. Cirrhosis can be due to fatty liver, alcohol abuse, hepatitis B and C infections, Wilson’s disease, cystic fibrosis, hemochromatosis, primary sclerosing cholangitis, biliary atresia, and schistosomiasis.
• Prehepatic hypertension: The prehepatic causes are portal vein thrombosis and congenital portal vein atresia.
• Posthepatic hypertension: The posthepatic causes of portal hypertension are hepatic vein thrombosis, inferior vena cava thrombosis and restrictive pericarditis.

Symptoms

Some common symptoms of portal hypertension are:

• Varices: Blood gets redirected and gathers into other veins making its way to the heart. The veins are swollen and enlarged.
• Esophageal gastric varices: This can be life threatening with symptoms of hematemesis, tarry, and bloody stools.
• Hepatic encephalopathy: The liver is unable to filter waste products and these get accumulated, causing lethargy and confusion.
• Ascites: Due to decrease of protein in the body, there is abnormal fluid collection within the peritoneum.
• Splenomegaly: Blood and blood components are trapped in the spleen causing enlargement of spleen.

Treatments

In cases where a shunt is not possible, then devascularization is done. This involves removal of the bleeding varices.

The fluid in the abdomen may be removed surgically by paracentesis.

As a last option, a liver transplant surgery may also be considered.

Extrahepatic portal venous obstruction (EHPVO)

Extrahepatic portal venous obstruction (EHPVO) is a common cause of major and life threatening upper gastrointestinal bleeding among Indians. Patients have large spleens and stunted growth. The diagnosis is easily confirmed by Doppler ultrasonography. Endoscopy sclerotherapy is the best option for the control of acute variceal bleeding.

Surgical intervention for portal hypertension is considered only when methods to control varices such as sclerotherapy, latex banding and balloon tamponade fail to control the bleeding. There are at least two decompression procedures that can be considered:

• Shunt surgery: In this surgery, an alternative pathway is created for flow of blood by artificially connecting a branch of portal vein to the renal vein.
• Transjugular intrahepatic portosystemic shunt (TIPS) A tubular connection is placed within the liver using x-ray guidance, joining two veins in the liver. This is commonly called a stent. The procedure is usually done under general anaesthesia.
• Orthotopic liver transplantation (OLT) The last option for treatment is liver transplantation which can be living donor or cadaveric liver transplant.

NON-CIRRHOTIC PORTAL FIBROSIS (NCPF)

Non-cirrhotic portal fibrosis (NCPF) is a chronic liver disease and type of non-cirrhotic portal hypertension (NCPH). Non-cirrhotic portal hypertension (NCPH) is a term used to describe a heterogeneous group of diseases characterized by the presence of portal hypertension with preserved liver structure and function. When there is an obstruction in the free flow of blood in the liver, it increases the pressure and is known as Non-cirrhotic portal fibrosis This increased blood pressure stresses the blood vessels in the esophagus, stomach, and other digestive organs and results in internal bleeding.