Treatment

The aim of treatment for Budd-Chiari syndrome to keep your liver function stable by maintaining the flow of blood out of the liver. It very important that all patients with Budd-Chiari syndrome are treated in specialized hospitals with the required expertise, by a multidisciplinary team. This team of doctors includes liver specialists, blood disorder specialists (hematologists), and radiologists. They will look to:

• re-channel the blocked veins if possible.
• prevent recurrence or progression of thrombosis by thinning the blood.
• 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
• treat the underlying disease that caused the thrombosis in the first place.

Doctors will try to pinpoint the exact area where blood flow obstructed to help them understand how your health may affect. The severity of Budd-Chiari syndrome can depend on where the clot located and the number of veins that are affected.

Interventional radiologists

Interventional 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 cacography used. 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’, then injected through the catheter to light up the blood vessels to make it easier to see in the X-ray. In some cases, the scan shows that only the end portion of the vein blocked and that much of the vein remains clear. In other cases, the vein more blocked and doctors will need to get to the vein via a tube put into the liver from the tummy.

Venoplasty or angioplasty:

Once the tube gets to the blockage, doctors can remove the clot and open the vein. This is called venoplasty or angioplasty. The vein opened with balloons and the interventional radiologist often decides to place a metal spring (stent) within it to keep it open.

Thrombolysis:

If the clot within the hepatic veins formed recently and difficult to remove, the catheter tube may be left in the hepatic vein for a day or two to allow clot-busting drugs (thrombolysis) to get rid of the clot. When venoplasty/stent succeeds it usually leads to rapid improvement in the patient’s overall condition.

TIPPS (stent in the veins):

In some patients, the blockages are too extensive for venoplasty to work. Doctors will look at which treatments are suitable for the symptoms, depending on how severe they are. When ascites or bleeding varies troublesome, a radiological procedure called TIPSS may offere. This lowers the 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 done using a needle guided by a catheter inserted through a tiny puncture in your neck. This procedure may performed either under general anesthesia or with sedation.

Surgical shunts:

Occasionally another operation may offer which reverses the flow in the portal vein so that it 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 or an artificial graft to make a new connection which allows the blood to escape from the congested liver. Followings shunts can create

Porto-caval shunt

Meso-caval shunt

These surgical procedures and TIPSS can effective, however, there a risk they can create an additional problem. The shunts mean that less blood goes through the liver to cleaned of toxins. As a consequence, there 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 treated using laxatives such as lactulose, or antibiotics, to help your body remove these toxins. In very few cases the TIPSS stent can altered in size to try and reduce the encephalopathy.

Anticoagulation:

Most experts now recommend that patients with Budd-Chiari syndrome should receive life-long anticoagulation therapy because Budd-Chiari syndrome often means that people’s blood a tendency to clot too readily. Anticoagulation therapy involves taking medicines and being closely monitored with regular blood tests

Treatment of Myeloproliferative Neoplasia (MPN):

In patients with MPN, the hematologist will assess the patient’s disease type and the risk of further thrombosis in order to recommend the appropriate treatment. Assessment includes a molecular test from the blood for acquired mutations, such as JAK2, and a biopsy of the bone marrow. The treatment for MPN usually involves taking drugs long term to control the activity of the bone marrow and blood counts. This type of treatment called cytoreductive therapy and consists of either taking tablets or injections, which require monitoring on a regular basis.

Liver transplantation:

A liver transplant is usually only recommended if other treatments no longer helpful and your life threatened by end-stage liver disease. In Budd-Chiari syndrome, a liver transplant may require when:

• an onset of fulminant Budd-Chiari syndrome causes your liver to fail.
• your liver stops performing all of its functions adequately, a condition called decompensated cirrhosis.
• shunt procedures cannot prevent further deterioration in your condition.

A major operation and if it, 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.

The prognosis (Outlook)

Being diagnosed with Budd-Chiari syndrome can worrying and frightening, as it a serious disease. Diagnosis also gives patients an opportunity to have an effective treatment that can improve their quality of life. Treatments aim to reduce the risk of the disease progressing to a serious outcome and to improve the quality of life. How well you respond to treatment will depend on your age and the severity of the disease at diagnosis. Over 80% of patients do respond well and still alive 10 years after diagnosis. You should speak to your own doctor about your prognosis.