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Noncirrhotic Portal Hypertension Symptoms and Treatment Guidelines

What causes non-cirrhotic portal hypertension? Can you have cirrhosis without portal hypertension? Let’s find out about noncirrhotic portal hypertension symptoms and treatment!

Noncirrhotic Portal Hypertension Definition

Noncirrhotic portal hypertension meaning – What is noncirrhotic portal hypertension? Noncirrhotic portal hypertension is a type that doesn’t have cirrhosis. Only 10% of people with portal hypertension have cirrhosis.

noncirrhotic portal hypertension symptoms and treatment guidelines - difference between cirrhotic and non cirrhotic portal hypertension

Noncirrhotic Portal Hypertension Causes

What causes noncirrhotic portal hypertension? What is the most common cause of noncirrhotic portal hypertension?

Noncirrhotic portal hypertension can be caused by extrahepatic portal vein obstruction (portal vein thrombosis often with cavernous transformation [portal cavernoma]), splenic vein obstruction (presenting as gastric varices without esophageal varices), schistosomiasis, nodular regenerative hyperplasia, and arterial-portal vein fistula. Idiopathic noncirrhotic portal hypertension is common in India. It has been linked to chronic infections, exposure to medications or toxins, prothrombotic disorders, immunologic disorders, and genetic disorders that cause obliterative vascular lesions in the liver.

It is rare in the West, where the death rate is higher because of other diseases and older age. It has been called portosinusoidal vascular disease.

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Portal vein thrombosis can happen in 10–25% of people with cirrhosis. It is linked to the severity of liver disease and is partly caused by acquired protein C deficiency and splenorenal shunts, which stop blood flow through the portal veins. It may be linked to hepatocellular carcinoma but not to a higher risk of death. Oral contraceptives, pregnancy, chronic inflammatory diseases (like pancreatitis), damage to the portal venous system (like surgery), other types of cancer, and the use of eltrombopag to treat thrombocytopenia are also risk factors.

Portal vein thrombosis can be put into three types: type 1, which affects the main portal vein; type 2, which affects one (2a) or both (2b) of the portal vein’s branches; and type 3, which affects both the main portal vein and its branches. Other ways to describe a liver disease are whether it is occlusive, how long it has been going on, and whether it has spread into the mesenteric vein. Pancreatitis or pancreatic cancer can be made worse by splenic vein thrombosis.

Pylephlebitis, or septic thrombophlebitis of the portal vein, can be a complication of appendicitis or diverticulitis, especially when anaerobic organisms, like Bacteroides species, are involved. Nodular regenerative hyperplasia happens when the blood flow to the liver is changed. It can be caused by collagen vascular diseases, myeloproliferative disorders, and drugs like azathioprine, 5-fluorouracil, oxaliplatin, and thioguanine. Some cases of noncirrhotic portal hypertension, which is often caused by nodular regenerative hyperplasia, have been linked to the long-term use of didanosine or a combination of didanosine and stavudine in HIV patients. Genetics may also play a role.

Obliterative portal venopathy is the primary blockage of intrahepatic portal veins without cirrhosis, inflammation, or hepatocellular neoplasia.

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Noncirrhotic Portal Hypertension Diagnosis

  • Splenomegaly or bleeding from the esophagus or stomach varices in people who don’t have liver disease.
  • Cirrhosis is made worse by a blood clot in the portal vein.

Noncirrhotic Portal Hypertension Symptoms

Signs and symptoms of noncirrhotic portal hypertension: What are some symptoms of noncirrhotic portal hypertension?

Most of the time, abdominal pain is caused by acute portal vein thrombosis. Aside from a big spleen, the physical symptoms aren’t anything special. However, hepatic decompensation can happen after severe gastrointestinal bleeding. A liver disorder or intestinal infarction can happen simultaneously when portal vein thrombosis and mesenteric vein thrombosis happen together. In addition, 25% of people with noncirrhotic portal hypertension can get ascites. Covert hepatic encephalopathy is common in people with portal vein thrombosis but who don’t have cirrhosis.

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Laboratory Findings

Most of the time, the results of biochemical tests on the liver are normal, but there may be signs of hypersplenism. Many people with portal vein thrombosis have an underlying condition that makes them more likely to bleed. These conditions include myeloproliferative neoplasms (often linked to a specific mutation [V617F] in the gene coding for a JAK2 tyrosine kinase, which is found in 24% of portal vein thrombosis cases), mutation G20210A of prothrombin, factor V Leiden mutation, protein C and S

It is possible, though, that in many cases, signs of hypercoagulability are just a side effect of portosystemic shunting and less blood flow to the liver.


Most of the time, colour Doppler ultrasonography is the first test used to diagnose portal vein thrombosis. The portal system’s contrast-enhanced CT or magnetic resonance angiography (MRA) is usually conclusive. It can measure the spread of thrombus into the mesenteric veins and rule out tumour thrombus in patients with cirrhosis. EUS might be useful in some situations.

Magnetic resonance cholangiography can show that a large portal cavernoma (portal biliopathy) is pressing on the bile duct in a person with jaundice. This may be more common in people with an underlying hypercoagulable state than in people without one. When a person has pylephlebitis, a CT scan may show an infection source inside the abdomen, thrombosis or gas in the portal vein system or a hepatic abscess.

Other Studies

Endoscopy shows varices in the esophagus or stomach. Needle liver biopsy may be used to diagnose schistosomiasis, nodular regenerative hyperplasia, and noncirrhotic portal fibrosis, and it may show sinusoidal dilatation. In addition, by measuring how stiff the liver is with elastography, doctors can tell the difference between noncirrhotic portal hypertension and cirrhosis.

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Noncirrhotic Portal Hypertension Treatment and Management Guidelines

Treatment of noncirrhotic portal hypertension – What is the best treatment for noncirrhotic portal hypertension?

A splenectomy will stop the bleeding if a clot causes bleeding from the varix in a splenic vein. For other causes of noncirrhotic portal hypertension, such as variceal bleeding, band ligation and betablockers are used to lower portal pressure. Portosystemic shunting (including TIPS) is only done if endoscopic therapy fails. Rarely is liver transplantation needed for progressive liver dysfunction.

Anticoagulation, especially with low-molecular-weight or unfractionated heparin or thrombolytic therapy, may be recommended for isolated acute portal vein thrombosis (and leads to at least partial recanalization in up to 75% of cases when started within 6 months of thrombosis) and possibly for acute splenic vein thrombosis. An oral anticoagulant is continued long-term if a hypercoagulable disorder is found or if an acute portal.

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For a person with cirrhosis and portal vein thrombosis, whether or not to give them an anticoagulant depends on whether or not they have ascites, how likely they are to fall, and whether or not they can get a liver transplant.

Also, in 30–50% of cases, partial portal vein thrombosis can improve. Unfortunately, there isn’t much information about how patients with cirrhosis and portal vein thrombosis should use direct-acting oral anticoagulants.

Patients with cirrhosis may benefit from using enoxaparin to prevent portal vein thrombosis and hepatic decompensation.

Patients with portal hypertension who don’t have cirrhosis should be sent to a specialist.

I hope you understand noncirrhotic portal hypertension symptoms and treatment guidelines.

About Micel Ortega

Dr. Micel Ortega, MD, PhD, is a highly respected medical practitioner with over 15 years of experience in the field of internal medicine. As a practicing physician, Dr. Micel has built a reputation for providing compassionate and evidence-based care to his patients. He specializes in the diagnosis and management of chronic conditions, including diabetes, hypertension, and heart disease. In addition to his clinical work, Dr. Micel has published extensively in top-tier medical journals on the latest advancements in internal medicine and has played an instrumental role in the development of innovative treatment options.

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