In this condition there is obstruction to the venous outflow of the liver owing to occlusion of the hepatic vein.
In one-third of patients the cause is unknown, but specific causes include hypercoagulability states, such as polycythaemia vera, taking the contraceptive pill, or leukaemia.
Other causes include occlusion of the hepatic vein owing to posterior abdominal wall sarcomas, renal or adrenal tumours, HCC, hepatic infections (e.g. hydatid cyst), congenital venous webs, radiotherapy, or trauma to the liver.
The acute form presents with abdominal pain, nausea, vomiting, tender hepatomegaly and ascites. The liver histology shows centrilobular congestion with hepatocyte atrophy. In the chronic form there is enlargement of the liver (particularly the caudate lobe), mild jaundice, ascites, a negative hepatojugular reflex, and splenomegaly with portal hypertension.
Investigations show a high protein content in the ascitic fluid and characteristic liver histology. Ultrasound, CT or MRI will demonstrate hepatic vein occlusion and an enlarged caudate lobe which has a different venous drainage;
There may be compression of the inferior vena cava. Venography may demonstrate the thrombosed vein. Pulsed Doppler sonography or a colour Doppler are useful as they show abnormalities in the direction of flow in the hepatic vein.
Ascites should be treated as well as any underlying cause, e.g. polycythaemia. Congenital webs should be resected surgically. A side-to-side portocaval or splenorenal anastomosis may decompress the congested liver, with considerable improvement in the clinical state of the patient. A peritoneal-jugular LeVeen shunt for resistant ascites is occasionally useful. Liver transplantation is becoming the treatment of choice.
A similar clinical picture can be produced by inferior vena caval obstruction, right-sided cardiac failure or constrictive pericarditis, and appropriate investigations should be performed.
The prognosis depends on the aetiology, but some patients can survive for several years.