Budd-Chiari syndrome (BCS) is a disorder affecting the liver and blood vessels. Where blood flowing into the liver has difficulty in being able to flow out. leading to serious complications. Budd-Chiari Syndrome takes its name from George Budd. The English doctor who described the first cases in 1845, and Hans Chiari. The Austrian pathologist who credited with providing the first description of how it affects the body in 1899.
Blood flows into the liver from two blood vessels – the portal vein and the hepatic artery. After the blood has passed through the liver. it flows out through the hepatic veins and into the inferior vena cava. a large blood vessel that carries blood back to the heart. In Budd-Chiari syndrome, this flow partially blocked.
The immediate result of a build-up (congestion) blood in the liver. There is more flowing in than flowing out. The liver becomes swollen also tender to the touch and a source of discomfort. The congestion causes fluid to leak from the liver into the abdominal cavity. The resulting build-up of fluid in the abdominal area called ascites. One of the commonest symptoms of Budd-Chiari syndrome. It may visible as a bulge in your tummy area and can uncomfortable and make. it hard to breathe and eat normally.
If the blockage extensive and also affects the inferior vena cava. There may other places that become swollen. This is particularly likely in the ankles and legs and called peripheral oedema.
DIAGNOSIS OF BUDD CHIARI SYNDROME:
Including LFT’s, CBC, Clotting profile and also a blood test to identify the underlying blood disorder.
Ultrasound directs sound waves through your skin via a probe device as it is passed over your liver area. In Budd-Chiari syndrome it used to obtain information about blood flow in your arteries and veins and this often gives enough information for the condition to diagnosed.
A CT or MRI scan:
A CT or MRI scan may also be used. This can obtain pictures from different angles. around your body using computer processing and can also show cross-sections of your tissue and organs. This scan can show an enlargement of the liver and changes in the density of liver tissue due to abnormal blood flow. A liver biopsy may necessary if other tests have helped doctors to identify the reason for liver problems.
The aim of treatment for Budd-Chiari syndrome is 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 treated in specialised hospitals with the required expertise, by a multidisciplinary team. This team of doctors includes liver specialists, blood disorder specialists (haematologists), and radiologists. They will be looking 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 affected. The severity of Budd-Chiari syndrome can depend on where the clot is located and the number of veins that affected.
Interventional radiologists will use X-ray techniques known as hepatic angiography or venography. 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. These procedures involve inserting a thin and flexible tube (catheter) into a blood vessel. and 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 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.
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 is opened with balloons and the interventional radiologist often decides to place a metal spring (stent) within it to keep it open.
If the clot within the hepatic veins was formed recently and difficult to remove. The catheter tube may 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 and depending on how severe they are. When ascites or bleeding varices are troublesome. a radiological procedure called TIPSS may be offered. This lowers the pressure in the portal vein. In this procedure, a metal or plastic tube (stent) 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 procedure may performed either under general anaesthesia or with sedation.
Occasionally another operation may offer which reverses the flow in the portal vein. so that it took blood out of the liver rather than into it. 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 also to escape from the congested liver. Followings shunts can be created.
These surgical procedures and TIPSS can effective. however, there is a risk they can create an additional problem. shunts mean that less blood and goes through the liver to cleaned of toxins. As a consequence, there is a risk these toxins will build up and this can cause a condition called hepatic encephalopathy. 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 altered in size to try and reduce the encephalopathy.
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 has 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 haematologist 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 also usually involves taking drugs long term to control the activity of the bone marrow and blood counts. This type of treatment is called cytoreductive therapy and consists of either taking tablets or injections. which require monitoring on a regular basis.
A liver transplant 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 be required 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.
Liver transplantation a major operation and if not an emergency treatment. you will need to plan it carefully with your medical team also family and friends. Liver transplants offer a good prospect of a full recovery.
The prognosis (Outlook)
Being diagnosed with Budd-Chiari syndrome can also worrying and frightening. as it is a serious disease. Diagnosis also gives patients and, an opportunity an effective treatment that can improve the quality of life. Treatments aim to reduce the risk of the disease and 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 also about your prognosis.