Badda-Chiari syndrome- pathology that disrupts the outflow of blood from the liver .It arises from the primary obliterating endophlebitis of the hepatic veins and their thrombosis.
Disease is a secondary disorder of blood outflow from the liver, with pathological conditions that are not associated with changes in the blood vessels of the liver.
The name of the disease comes from the names of doctors who described it for the first time( 1845 - English doctor Budd, 1899 - Austrian pathologist Chiari).
Contents of
What causes
- Treatment methods
- Purpose of treatment for complication of
What causes the disease
In order to understand the causethe emergence of thrombosis in the vessels of the liver, it is necessary to understand the process of the hepatic circulation.
Blood circulation in the liver is carried out by three systems:
- system responsible for blood flow to the lobules;
- system, which provides blood circulation inside the lobules;
- system designed for unobstructed outflow of blood from the lobules.
In the first system( bringing), the portal vein collects blood throughout the abdominal region and, through the hepatic artery from the aorta, delivers blood to smaller veins and arteries. They also permeate the segments and lobes of the liver, pass around and between them.
Around the lobular arteries and small vessels of the liver entering the lobes inside, form intralobular sinusoidal capillaries. Flowing sinusoidal capillaries blood enters the central vein and turns into venous.
Then it is sent again to the collecting( portal) vein, then to the hepatic veins that flow into the lower vena cava. This is how the outflow of blood occurs in the liver.
Disease, representing a danger to health and life, thrombosis of the cavernous sinus - methods of treatment and the necessary prevention of pathology are discussed here.
In the blood vessels of the liver, pathological changes may occur that trigger the development of the Budd Chiari syndrome.
The consequences of these changes are increased blood pressure and venous congestion in the abdominal cavity.
The result is an increase in the portal vein of the liver in its size and the appearance of symptoms of fibrosis:
- the liver becomes larger than its size so that it begins to extend beyond the edge of the edge arch;
- is the development of ascites( accumulation of a large amount of fluid in the abdominal cavity);
- , along with these processes, the spleen also increases;
- on the front side of the abdomen occurs varicose veins.
These pathological processes cause the development of obliterating phlebitis. But the main cause of the onset and acute course of this rare disease is rightly considered thrombosis of the hepatic veins.
The development of Buddha-Chiari's disease is caused by:
- received abdominal and abdominal injuries;
- impaired blood coagulability;
- presence of cirrhosis;
- complicated pregnancy and childbirth;
- prolonged use of hormonal drugs, in particular contraceptives;
- long-term use of medications that cause increased blood coagulability;
- various tumors developing in the abdominal cavity;
- is an autoimmune disease;
- surgical interventions( operations) in the abdominal region;
- toxic and other liver damage( focal);
- liver vessels with congenital anomaly;
- hollow vein thrombosis;
- increased number of erythrocytes in the blood;
- intravenous introduction of nutrients into the human body, which can not feed on its own;
- neoplasms of the heart, liver and adrenal glands;
- infectious diseases such as tuberculosis or syphilis.
Signs of the pathology of
The disease manifests itself in symptoms such as:
- in most patients( about 90%) there is an increase in the size of the stomach( accumulation of large amounts of fluid);
- increased sizes of spleen and liver;
- bleeding of the esophagus;
- peripheral edema and edema of the lower limbs( the cause is thrombosis);
- skin becomes yellowish;
- in 80% of the diseases shows abdominal pain, nausea and vomiting( perhaps even bloody);
- patient's stool disorder( in particular - diarrhea).
The situation is much more complicated when there is a little noticeable chronic development of the Badd-Chiari syndrome. Few people seriously draw attention and are alarmed by vomiting, painful sensations in the right hypochondrium and barely noticeable yellowing of the skin.
If you do not react to these symptoms, the acute development of the disease will begin, which leads to a disorder in the functions of the liver. There is a hepatic coma, which in most cases ends with a lethal outcome of
Death occurs almost instantly, the reason for this - the development of acute renal failure.
Diagnostic features and diagnosis of
The objectives of the diagnosis are as follows:
- reveal Badda-Chiari syndrome;
- if any, then check for possible complications;
- and finally - to determine what genesis of the disease( primary or secondary).
Diagnosis of the disease using such techniques:
- ultrasound of the abdominal cavity;
- tomography( magnetic nuclear, computer, magnetic resonance);
- for complete confirmation / disproving the Badd-Chiari syndrome is made by catheterization of the hepatic vein, then angiography is performed, it is also able to partially dilute the resulting thrombosis( this is very important at an acute stage of the syndrome development);
- appointment of an internal X-ray;
- biochemical and general blood test;
- histological examination of liver biopsy.
What you need to know about treating the pathology of
The goal of treating the disease becomes:
- to eliminate venous thrombosis and thereby reduce the manifestation of unpleasant symptoms of the disease;
- to carry out prevention and subsequent treatment of the syndrome;
- improve the patient's condition and give him hope and a chance to live.
Methods of treatment
Self-treatment with syndrome is excluded.
The main condition for a successful recovery is a stationary regimen and medication prescribed by the attending physician( if necessary, surgical intervention).
Thus, the methods of treating the disease are two:
- Medical therapy ( conservative) is only an auxiliary, preventive measure of treatment. It consists in taking medications to eliminate thrombosis and venous stasis. Such prophylaxis does not give a permanent result and a lasting effect and without a subsequent surgical intervention in most cases( 80-90%) there is a lethal outcome. Therapeutic drugs include diuretics, thrombolytic drugs and anticoagulants.
- Surgical intervention can be of several types. Determine what is necessary, in this or that situation can only be determined by the cause of the disease. Most often, procedures are performed bypass( mesoatrial, mesocaval, portocaval).In chronic conditions, the doctor prescribes the second and third type of bypass, and in the case of thrombosis, mesoatrial.
But none of these methods will yield results in the development of hepatic-cell carcinoma( especially severe form of the disease).
In this case only the donor transplant( transplantation) of the liver will help to survive for about five years.
This procedure will help in restoring the functionality of the patient's liver.
The purpose of treatment for the complication of the syndrome
Complications of the disease require special treatment, as it becomes necessary to combine the therapy of several types of already complicated diagnoses: cirrhosis, gastrointestinal bleeding, varicose veins of the esophagus, stomach and intestines.
In case of complications, the doctor treating prescribes beta-blockade therapy .
Forecast for recovery
Forecasts are disappointing.
Chances though small, but still there, if the timely start treatment.
Prevention of
The first and main rule of disease prevention is the elimination of all possible risk factors. To conduct the diagnosis at the slightest suspicion of the disease, to begin her treatment as early as possible - then there will be more chances for recovery.
It is better to prevent the disease than to treat it for a long time and in vain( in most cases).