Budd-Chiari syndrome is a disease that is associated with obstruction of blood vessels located in the liver. Due to the pathology, the blood flow is disturbed, and stagnation in the veins can occur. This problem is caused as a result of minor vascular diseases. The described syndrome is considered a rare pathology. It can occur in a patient in one of three forms: acute, subacute, and chronic. If we talk about the manifestations, the patient feels pain, nausea. There is jaundice and ascites. Vomiting may occur. On palpation, it is clearly noticeable that the liver is slightly enlarged. Treatment with medications practically does not give relief. To solve the problem, an operation is necessary. The patient’s future life depends on its result.
Description of the problem
Budd-Chiari syndrome causes portal hypertension, so it is important to start therapy on time. The problem occurs in 20-60% of cases from all pathologies associated with the liver. Its danger lies in the fact that over time, cells of the organ are damaged, respectively, it is not able to perform all functions. If you do not start treatment at an early stage, then the patient will become disabled or die. This is due to complications that affect other organs.
Most often, Budd-Chiari syndrome occurs in women, especially if there are genetic prerequisites. At risk are women aged 40-50 years. The disease is diagnosed in one person per thousand. Only 55% of patients survive. As a rule, death occurs due to renal failure.
Chiari Disease and Budd-Chiari Syndrome: Differences
Chiari's disease is characterized by the development of endophlebitis, which affects the hepatic veins. In a person, the outflow of blood due to thrombosis is impaired. Any abnormalities may also occur.
Syndrome is an inflammation characterized by impaired blood flow. The difference between this pathology and Chiari disease is that at the first there are no changes in the blood vessels of the liver that could lead to the described problem.
As mentioned above, Budd-Chiari syndrome (a photo of external manifestations is available in the article) is quite rare. It is detected on autopsy with a frequency of up to 0.006%. Chiari's disease is even less common. Both pathologies are not completely cured, you can only try to maximize the life of the patient.
Causes of occurrence
Budd-Chiari syndrome is often the result of any serious congenital malformations. Especially if they are associated with the liver or blood vessels. Consider in more detail the factors that can provoke the disease.
- Cirrhosis of the liver.
- Migratory thrombophlebitis.
- Various neoplasms.
- Infections that have become chronic.
- Hematology problems. They can be either congenital or acquired. It should highlight intestinal diseases, anemia, and so on.
- Mechanical factors. This may be obstruction that occurs after surgery or injury, problems with veins in the liver (narrowing), and more.
Disease classification
When treating Budd-Chiari syndrome, it is important to understand what type of pathology is inherent in a particular patient. In 30% of cases, an idiopathic problem occurs. In 20% of all diseases, pathology was provoked by disorders of blood coagulation, and in 9% - malignant tumors.
If we talk about the etiological causes of the syndrome, then the disease can be divided into several types. Let's consider them further.
- Idiopathic (the cause of the pathology is unknown).
- Appeared as a result of oncopathology.
- Arisen due to cirrhosis.
- Congenital (develops due to stenosis of a vein in the liver).
- Post-traumatic (diagnosed due to trauma, phlebitis, and so on).
It is these reasons that can cause thrombosis, stenosis and other vascular diseases, which cause the Budd-Chiari syndrome. Symptoms of the pathology are rather unpleasant (we will talk about them later). However, in many cases, all manifestations are similar to each other. For example, most of the causes can cause hepatomegaly. Because of it, the liver capsule begins to stretch very much, and the person feels pain. Unpleasant sensations are localized in the right hypochondrium.
In the predominant part of patients, lymph withdrawal begins not through the affected vein, but due to the intercostal vessels, as well as the paravertebral circulatory system. However, this still does not allow for complete drainage and stagnation occurs in the patient. This leads to the development of atrophy and hypertrophy of some parts of the liver. In almost half of patients, obstruction of the inferior vein occurs. This is due to an increase in the size of the caudate lobe. This over time leads to failure. This pathology is caused by cirrhosis of the liver, paresis of vessels or necrosis.
Three types of Budd-Chiari syndrome are distinguished, depending on the place of thrombosis.
- Hepatic vein enlargement.
- The growth of large vessels in the organ.
- The growth of small vessels.
Symptomatology
How this disease manifests itself depends on the location of those vessels that have become clogged. Also, the symptomatology varies from the type of pathology and from whether there are other problems in a person.
Most often, the syndrome in the early stages does not occur at all. It can be detected accidentally during ultrasound, x-ray and tomography. Over time, nausea, vomiting, hepatomegaly, as well as compaction of the liver itself will occur.
In some patients, veins located on the abdomen and chest can expand. They can be seen at rest during an external examination. The late stages of the problem are manifested by the occurrence of liver failure and hypertension.
In 15% of all cases, the patient has pain, which over time begins to increase, jaundice, vomiting, enlargement of the affected organ, expansion of the veins, as well as swelling of the lower extremities.
Like Chiari disease, Budd-Chiari syndrome progresses very quickly. If an acute form occurs, after a few days the patient begins complications in the form of ascites. Moreover, it is impossible to correct or eliminate it with the help of diuretic drugs. Late stage occurs in 20% of patients. It is characterized by expansion of veins not only in the liver, but also in the stomach. Encephalopathy also occurs.
There is a fulminant form of the syndrome, but it is diagnosed quite rarely. It is characterized by almost instant (within a few days) development of the disease.
If you ignore adequate surgical and medical treatment, then a person will begin to experience complications of various types. Budd-Chiari syndrome causes portal hypertension, so the patient will experience bleeding in the esophagus and stomach, hypersplenism and ascites. The second disease is characterized by enlarged spleen, in which the body eliminates most blood enzymes.
A huge number of patients suffering from obstruction of the veins die from renal failure in just three years.
Emergency
Sometimes the described pathology can be complicated by bleeding and impaired urination. In order to get the right help, you should immediately call an ambulance. The characteristics of the emergency are: tarry stools, a decrease in the amount of urine, an increase in all symptoms, and also vomiting, which takes on the color of coffee.
Diagnostic measures
Diagnosis of Budd-Chiari syndrome is carried out using special liver tests, which can also detect the presence of thrombosis. A biochemical blood test should be done, which will make it clear the condition of the diseased organ as a whole.
To clarify the assumptions, the patient must undergo an ultrasound of the abdominal cavity and liver, computed tomography and magnetic resonance imaging. Cavagraphy and venohepatography also do not interfere.
If the results are uncertain, then the doctor may prescribe a biopsy. Thanks to it, it will be possible to identify the presence of blood stasis, thrombosis and atrophy of enzymes.
Therapeutic measures
To cure Budd-Chiari syndrome, you need to contact a specialist. Therapy can be performed by a surgeon and a gastroenterologist. Be sure to have to go to hospital. If there are no signs of liver failure, then an operation is performed. During it, compounds are applied that will be localized between the vessels in the liver. One of three types of operations can be performed:
- shunting;
- angioplasty;
- dilatation.
If the patient has venous stasis or vein fusion, then surgery is performed. During it, vasodilation occurs. In cases where there is cirrhosis of the liver or other consequences that cannot be eliminated, the doctor may prescribe a transplant of a diseased organ.
Preparation for surgery
When the doctor prepares the patient for surgery, he must be sent for an ultrasound scan. Budd-Chiari syndrome is a disease that has severe consequences, so it is important to know all the nuances. Before and after surgery, medications are prescribed. They are necessary in order to restore blood flow in the affected organ. Drugs that relieve symptoms may also be prescribed. After a certain period, the patient should be tested for electrolyte levels. If necessary, therapy may be slightly adjusted. In the treatment of glucocorticoids, thrombolytics, diuretics and those drugs that normalize the metabolism and improve liver functionality.
It should be understood that conservative therapy in the development of Budd-Chiari syndrome is not highly effective and cannot replace surgery. Medicines will only achieve a temporary result. Those patients who refused surgery in 85% of cases die within 2 years.
Forecast
The prognosis for the development of this syndrome is unfavorable. It is impossible to recover completely. In 20% of cases, according to statistics, they progress so quickly that the patient falls into a coma within a few days and then dies.
If the disease proceeds in a chronic form (85%), then the symptoms gradually increase. Pathology can begin to progress in at least a month, a maximum of six months. Cirrhosis or insufficiency occurs, so the patient dies within 2-5 years.
Life extension
Thanks to medical treatment, the patient can extend the life of no more than 2-3 years. During various operations, such as shunting or liver transplantation, the patient's condition improves greatly. After the intervention, the patient can live 10-20 years.