The aim of treatment for BCS is to keep your liver function stable by maintaining the flow of blood out of the liver. Doctors will be looking to:
- re-channel the blocked veins if possible
- prevent recurrence or progression of thrombosis
- ease or ‘decompress’ the congestion of blood in your liver
- manage your ascites
- prevent further damage to your liver and allow liver cells to regenerate.
Doctors will try to pinpoint the exact area where blood fl ow is obstructed to help them understand how your health may be affected (your ‘prognosis’). The severity of BCS can depend on where the clot is located and the number of veins that are affected.
Radiologists will use X-ray techniques known as hepatic angiography or venography to examine arteries and veins directly to determine the location and severity of a clot. If the inferior vena cava is being examined, an X-ray called inferior cavography is used. This is usually done under local anaesthetic and you are also likely to be given sedation. It is possible you may be asked to stay in hospital overnight.
These procedures involve inserting a thin, flexible tube (catheter) into a blood vessel through an easily accessible vein in the arm, neck or groin. A dye, referred to as a ‘contrast dye’ or ‘contrast medium’, is then injected through the catheter to light up the blood vessels to make them easier to see in the X-ray. In some cases the scan shows that only the end portion of he vein is blocked and that much of the vein remains clear. In other cases, the vein is more blocked and doctors will need to get to the vein via a tube put into the liver from the tummy.
Once the tube gets to the blockage, doctors can remove the clot and open the vein. This is called venoplasty or angioplasty. The vein is opened with balloons and the doctor may decide to place a metal spring (stent) within it to keep it open. If the clot within the hepatic veins was formed recently and is diffi cult to remove, the catheter tube may be left in the hepatic vein for a day or two to allow clotbusting drugs (thrombolysis) to get rid of the clot. When venoplasty succeeds it usually leads to rapid improvement in the patient’s overall condition.
In some patients, the blockages are too extensive for venoplasty to work. Doctors will look at what treatments are suitable for the symptoms, depending on how severe they are. When ascites or bleeding varices are troublesome, a surgical procedure called TIPSS may be offered. This lowers pressure in the portal vein. In this procedure a metal or plastic tube (stent) is passed across your liver to make a shunt, or bypass, to make your blood travel straight from the portal vein past the blocked hepatic veins into the inferior vena cava which carries the blood back to the heart.This is done using a needle guided by a catheter inserted through a tiny puncture in your neck. This is not painful in itself but you will be given a local anaesthetic and usually some sedation.
Occasionally another operation may be offered which reverses fl ow in the portal vein so that it is taking blood out of the liver rather than into it. The liver still receives enough blood from the hepatic artery to function adequately. This operation uses a vein from the neck to make anew connection which allows the blood to escape from the congested liver. The vein from the neck is grafted on between the mesenteric vein, a vein from which blood normally flows into the portal vein and the inferior vena cava, and is called a meso-caval shunt.
These surgical procedures can be effective, however, there is a risk they can create an additional problem. The shunts mean that less blood goes through the liver to be cleaned of toxins. As a consequence, there is a risk these toxins will build up and this can cause a condition called hepatic encephalopathy. The symptoms of this can include mental confusion, tremors or drowsiness. Hepatic encephalopathy can be treated using laxatives such as lactulose, or antibiotics, to help your body remove these toxins.
Where membranous webs are the cause of BCS, angioplasty may be used to relieve the obstruction. This is a technique in which a catheter with a small balloon at the end is inserted into the blocked artery. The balloon is then infl ated to widen the artery and allow the blood to fl ow more freely. This may have to be carried out in a number of blood vessels.
Anticoagulation
Most experts now recommend that patients with BCS should receive life-long anticoagulation therapy, because BCS often means that people’s blood has a tendency to clot too readily. Anticoagulation therapy involves taking medicines and being closely monitored with regular blood tests to check that the levels of clotting are right, to make sure the blood does not clot too readily (which could cause thromboses or blockages) or not well enough (which could cause bleeding).
Liver transplantation
A liver transplant is usually only recommended if other treatments are no longer helpful and your life is threatened by end stage liver disease. In BCS, a liver transplant may be required when:
- an onset of fulminant BCS causes your liver to fail
- your liver stops performing all of its functions adequately, a condition called decompensated cirrhosis
- shunt procedures cannot prevent a further deterioration in your condition.
Liver transplantation is a major operation and if it is not an emergency treatment, you will need to plan it carefully with your medical team, family and friends. Liver transplants offer a good prospect of a full recovery.