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Budd Chiari Syndrome and Splanchnic Vascular Disorders

Vascular disorders of the liver are rare and often affect young adults. One of these is Splanchnic Vein Thrombosis which includes Budd–Chiari syndrome (BCS).

BCS is defined as hepatic venous outflow obstruction at any level from the small hepatic veins (HV) to the junction of the inferior vena cava (IVC) and the right atrium, regardless of the cause of obstruction.

BCS is a rare disorder affecting one in a million people and may result in liver failure. The most common underlying cause is a pro-thrombotic haematological condition, which is present in most patients. Myeloproliferative neoplasms (MPN) are the most common of these haematological disorders. Therefore, involvement of a haematologist is essential for long term treatment of the underlying myeloproliferative disease. Patients are anticoagulated and often prescribed powerful cytoreductive drugs to control the bone marrow disease.

Further management depends on the location of the hepatic outflow obstruction and the extent of hepatic dysfunction with attempts made to overcome the obstruction. This may involve interventional radiology (IR) and rarely surgery such as liver transplantation. Splanchnic Vein Thrombosis may also involve the portal venous system supplying blood to the liver. The treatments are broadly similar to BCS.

Other vascular disorders of the liver include idiopathic non-cirrhotic portal hypertension (INCPH) which often presents with a variceal haemorrhage and enlarged spleen. INCPH is also rare and patients are often very well with no symptoms, but at risk of bleeding from varices. The key to management is confident diagnosis and therapies to prevent variceal bleeding.

Due to the rarity of the above conditions, which often pose diagnostic and treatment challenges, all patients with these conditions should be managed in specialised units. The specialist clinic in the Centre for Rare Diseases in the only dedicated clinic in the UK for such liver disorders.

Information about the service

The monthly clinic run by Professor Dhiraj Tripathi, Consultant Hepatologist and Dr Hayder Hussein, Consultant Haematologist offers a one stop service for patients with BCS or other vascular liver disorders.

Consultations involve a detailed clinical assessment with blood tests including specialised molecular and haematological tests. Many patients also have a liver ultrasound scan at the same time, plus advice from interventional radiologists Dr Homoyon Mehrzad and Dr Salil Karkhanis.

Patients who have undergone radiological treatment will be offered follow up procedures at Queen Elizabeth Hospital Birmingham (QEHB) to ensure continuity of care.

Clinical trial patients will be seen by a research nurse. There are currently plans to appoint a dedicated nurse specialist to facilitate the overall care of patients.

How to contact the service

Surya Begum, Medical Secretary

Tel: 0121 371 4672

Angel Woodcock, Medical Secretary

Tel: 0121 371 4383

Last reviewed: 07 June 2023