Budd-Chiari syndrome is a clinical image that is caused by the closing or the blockage of the hepatic veins. It occurs with a classical triad of pain in the abdomen, hepatomegaly and ascites. Instances of occlusions consist of thrombosis of the hepatic veins. It usually affects one out of every million people. This syndrome could be fulminant, chronic, acute, or even asymptomatic.
Most of the patients suffering from this syndrome possess a number of underlying symptoms and conditions like:
- Thrombotic Diathesis
- Polycythemia Vera
- Paroxysmal Nocturnal Hemoglobinuria
- Pregnancy, despite the use of powerful birth restricting pills
- Chronic Inflammatory Diseases
- Clotting Disorders
Causes of Budd-Chiari Syndrome
There can be a number of causes of the the syndrome; however, most of them are due to the disorders that result in the formation of blood clots within the body. Normally, there are various underlying problems that predispose blood clots in humans. However, at various instances, the main cause of the the syndrome has still not been identified. Fortunately, several researchers have found out a few conditions that may cause this particular syndrome. Some of these conditions are:
- Polycythemia, which is also known as the sickle cells disease
- Use of oral contraceptives
- Liver diseases, including cancer
- Phlebitis or even the inflammation of the veins
- Trauma of the liver and other liver diseases
- Autoimmune-mediated process
- Myeloproliferative disorder which is the growth of abnormal cells, especially in the bone marrow
- Chronic inflammatory diseases
- Inherited as well as acquired disorders related to blood clots
Any condition or disease that causes the obstruction of the hepatic venous is a common reason behind the syndrome.
Signs and Symptoms of Budd-Chiari Syndrome
This is an acute syndrome that presents itself with progressive and severe pain in the upper abdomen. It results in hepatomegaly that causes the liver to enlarge, and causes other diseases such as jaundice, ascites, and encephalopathy. Severe necrosis hepatic and acidosis lactic may also be present. The hypertrophy of the caudate lobe is present, many a times. Most of the patients have a very gradually building form of the syndrome, and which does not cause much pain. A system of the venous collateral can form around an occlusion, like a web of the spider, and which can be seen with imaging. Patients can also progress towards cirrhosis, exhibiting signs of liver failure as the disease progresses.
While on the other hand, the incidental search and diagnosis of silent and asymptomatic state may not be a reason of major concern and can easily be treated.
Various studies and researches have tried to predict the survival rate of the patients suffering from the Budd-Chiari syndrome. However, in general, almost two-thirds of the patients who suffer from the syndrome remain alive for at least ten years from the time of diagnosis. The necessary negative prognosis indicators include encephalopathy, ascites, elevated child pugh scores, enhanced prothrombin time, as well as altered levels of serum made up of different substances like creatinine, sodium, albumin and bilirubin. The survival of the patients also depends, to a very large extent, on the underlying causes of the Budd-Chiari syndrome. For instance, patients with underlying myeloproliferative disorders may move towards severe leukemia and succumb to death.
Diagnosis of Budd-Chiari Syndrome
An internist, a physician who specialises in diagnosing the diseases related to the internal parts of the human body; a gastroenterologist, a physician specialising in the disorders of the digestive systems; or general surgeons, may diagnose the syndrome. Generally, doctors suspect cirrhosis as one of the major reasons for the symptoms of the syndrome in patients. While conducting the physical examinations, doctors would come to know about the enhanced size of the liver. Further tests might include:
- Blood tests for ascertaining the functioning of the liver
- Ultrasound liver tests that bring to light the enlarged size of the liver
- CT Scans and an MRI of the abdomen for detecting similar kinds of abnormalities
Once the abnormalities are discovered, additional tests may be needed.
While conducting a biopsy of the liver, the doctors remove a very small sample of the liver tissues for further rounds of tests. The tissues that are damaged by the syndrome can be very easily detectable by a doctor during such tests.
Some patients may be treated with dosages of sodium, and diuretics for controlling ascites. Anti-coagulants like heparin and warfarin can also be used for preventing the symptoms from aggravating any further. Most patients may need further diagnosis to chalk out the right kind of treatment. Mild forms of the syndrome can be treated with surgeries involving the creation of medical shunts, in order to divert the flow of blood around the obstructed part of the liver. Shunts should be placed right after the diagnosis. If the hepatic venous is blocked, the portal venous may be reached via the intra-hepatic parts of the inferior venous cava, via a process known as DIPS. Patients with stenosis or caval vena obstructions may benefit from an angioplasty surgery. The restricted research activities on thrombolytic drugs, along with the directly infused urokinase drug, and the plasminogen tissue activators, have shown moderate progress as far as treating the syndrome is concerned.
The surgery involving the transplantation of the liver is a very effective and a powerful way of treating the syndrome, which is performed world over by renowned physicians and surgeons. It is most commonly reserved for patients who have not responded positively to most of the other treatments, and who have a life expectancy of less than a year. The chances of recovery or survival post the transplantation may be between 60 % and 87 %.