Pathologies

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Presents as acute abdominal pain or upper gastrointestinal bleeding without underlying cirrhosis.

Venous thrombosis generally occurs due to blood stasis (by local factors) and prothrombotic disorders.

Local risk factors for portal vein thrombosis without cirrhosis are perinatal umbilical infections and pancreatitis. In the latter situation it leads to a thrombosis of the vena lienalis. It can also be provoked by a splenectomy.

The prothrombotic abnormalities can be acquired, among other things as a result of an underlying myeloproliferative disorder (detectable via JAK2 mutations). They can also be caused by congenital deficiencies, such as protein S deficiency, protein C deficiency, factor V Leiden and mutations in the prothrombin gene. An additional risk factor is oral contraception. In 50% of cases there is more than one risk factor. The clinic of an isolated portosplanchnic venous thrombosis is a coincidental finding, in a third of cases abdominal pain (due to venous congestion) and signs of systemic inflammation and esophageal varices bleeding. Because the liver is also supplied by the hepatic artery, no ischemia of the liver occurs.

The diagnosis is made using a Doppler examination of the liver. An old thrombosis gives a cavernous transformation of the portal vein. Rarely, a portal cholangiopathy develops due to compression of the collaterals on the choledochus.

Since the systematic anticoagulation of these patients, the five-year survival rate has increased to over 90%. Mortality can occur due to an intestinal infarction, especially when the mesenteric vein is also thrombosed.

Screening for esophageal varices should be performed.

Presents as a sudden onset of painful enlarged liver and formation of ascites.

In these conditions there is a disturbed venous drainage of the liver due to compression thrombosis in the large hepatic vein.

The syndrome is usually caused by a thrombosis in the hepatic veins. In 80% of cases the cause is a consequence of prothrombotic abnormalities (acquired or congenital deficiencies, the same risk factors as for portal vein thrombosis). In 50% there are several factors that play a role simultaneously. Very rarely the venous drainage is obstructed by a web in the vena cava.

Budd-Chiari syndrome usually presents acutely, with sudden, severe and painful hepatomegaly and the development of ascites. In this situation, the three hepatic veins are usually thrombosed.

In 15% of cases, there is also a portal vein thrombosis.

The diagnosis is made by means of an ultrasound, a CT or an MRI scan, where the hepatic veins are no longer demonstrable, together with perfusion disorders in the liver. There is usually a hypertrophy of the lobus caudatus, because it has a separate blood drainage, which is less likely to thrombose. The swollen liver gives compression on the vena cava. The ascitic fluid shows a high protein and albumin content.

The patient must be anticoagulated for life, even in the presence of esophageal varices and also after liver transplantation. If a hepatic vein is still open, venous drainage can be improved by angioplasty or transjugular intrahepatic portosystemic shunt (TIPS). In some cases, urgent liver transplantation is necessary.

In these conditions, there is impaired venous drainage from the liver, due to occlusion in the small hepatic venules, by damage to the endothelium of the sinusoids. It occurs in a graft-versus-host disease after bone marrow transplantation, or in response to certain cytostatics.

It is clinically characterized by icterus, tender hepatomegaly and ascites. It has a poor prognosis.

This is a congenital familial disease (autosomal dominant with a prevalence of 1 to 2 in 10,000), in which arterio-venous shunts develop in the liver. This can cause local ischemia in the liver, mainly in the bile ducts and gallbladder. The large AV shunts in the liver can also lead to a 'high output failure' of the heart.

The disease is characterized by frequent nosebleeds and the characteristic vascular abnormalities of the lips, nose and fingertips.

In case of liver-biliary ischemia and/or to prevent heart failure, a liver transplant is indicated.

This condition is rare and has different names in the literature (hepatoportal sclerosis, non-cirrhotic portal fibrosis, incomplete septal cirrhosis, nodular regenerative hyperplasia).

As a result of phlebosclerosis of the portal vein, there is a perfusion disorder of the liver. It is usually associated with immunological and systemic diseases, use of medication such as azathioprine, HIV medication and thrombophilic diseases. Non-cirrhotic portal hypertension is frequently found in cystic fibrosis (as a result of precipitation of bile in the small bile ducts).

It gives portal hypertension which usually manifests itself in the form of a greatly enlarged spleen and esophageal varices. On an ultrasound there are signs reminiscent of cirrhosis such as noularity of the liver parenchyma, but an elastography measurement gives values ​​< 10 kPa.

An increased incidence of portal vein thrombosis is found. The short-term prognosis is good (normal liver synthesis capacity), but it can evolve into chronic liver failure and the need for liver transplantation.

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