Badda-Kiari's syndrome – a disease which is characterized by obstruction of veins of the liver causing violation of a blood-groove and venous stagnation. Pathology can be caused by primary changes in vessels and secondary violations of blood system. This quite rare disease which proceeds in a sharp, subsharp or chronic form. The main symptoms are severe pain in the right podreberye, the increased liver, nausea, vomiting, moderate jaundice, ascites. The sharp form of a disease leading to a coma or the death of the patient is most dangerous. Conservative treatment does not yield resistant results, life expectancy directly depends on successful surgery.
Badda-Kiari's syndrome is the pathological process connected with change of a blood-groove in a liver because of reduction of a gleam of the veins adjacent to this body. The liver performs a set of functions, normal work of an organism depends on its state. Therefore violation of blood supply affects all systems and bodies and leads to the general intoxication. Badda-Kiari's syndrome can be caused by secondary pathology of large veins which leads to stagnation of blood in a liver, or, on the contrary, primary changes of vessels connected with genetic anomalies. It is quite rare pathology in gastroenterology: statistically, the frequency of defeat is equal 1:100 000 population. In 18% of cases the disease is caused by hematologic violations, in 9% - malignant tumors. In 30% of cases it is impossible to reveal associated diseases. Most often pathology affects women of 40-50 years.
Reasons of a syndrome of Badda-Kiari
Development of a syndrome of Badda-Kiari it can be caused by a set of factors. Congenital anomaly of vessels of a liver and their structural components, and also narrowing and an obliteration of hepatic veins is considered the main reason. In 30% of cases it is impossible to find out the exact reason of this state, then speak about an idiopathic syndrome of Badda-Kiari.
Traumatic injuries of a stomach, liver disease can provoke emergence of a syndrome of Badda-Kiari, the inflammation of a peritoneum (peritonitis) and a pericardium (perikardit), malignant tumors, changes of haemo dynamics, a vein thrombosis, some medicines, infectious diseases, pregnancy and childbirth.
All these reasons lead to violation of passability of veins and development of developments of stagnation which gradually cause liver tissue destruction. In addition, intra hepatic pressure increases that can lead to necrotic changes. Of course, at an obliteration of large vessels the blood-groove is carried out at the expense of other venous branches (an unpaired vein, intercostal and paravertebralny veins), but they do not cope with such volume of blood.
As a result everything comes to the end with an atrophy of peripheral departments of a liver and a hypertrophy of its central part. Increase in the sizes of a liver aggravates even more a situation as it squeezes a hollow vein stronger, causing its full obstruction.
Symptoms of a syndrome of Badda-Kiari
Different vessels can be involved in pathological process at Badda-Kiari's syndrome: small and big hepatic veins, lower hollow vein. Clinical signs of a syndrome directly depend on the number of the damaged vessels: if only one vein suffered, then pathology proceeds asymptomatically and does not cause deterioration in health. But change of a blood-groove in two and more veins does not take place completely.
Symptoms and their intensity depend on the sharp, subsharp or chronic course of a disease. The sharp form of pathology develops suddenly: patients feel severe pain in a stomach and in the field of the right podreberye, nausea and vomiting. There is moderate jaundice and sharply the liver sizes increase (gepatomegaliya). If pathology affects a hollow vein, then puffiness of the lower extremities, expansion of hypodermic veins on a body is noted. In several days there are a renal failure, ascites and which difficult give in to therapy and are followed by bloody vomiting. As a rule, this form comes to an end with a coma and the death of the patient.
The subsharp current is characterized by increase in a liver, rheological violations (to be exact, strengthening of coagulability of blood), ascites, a splenomegaliya (increase in a spleen).
The chronic form can long not give clinical signs, the disease is characterized only by increase in the sizes of a liver, weakness and increased fatigue. Gradually there is a discomfort under the lower right edge and vomiting. At the height of pathology cirrhosis is formed, the spleen increases, the liver failure as a result develops. Chronic form of a disease is registered in most cases (in 80%).
In literature single stories of the lightning form which is characterized by bystry and progressive increase of symptoms are described. For few days jaundice, a renal and liver failure, ascites develops.
Diagnostics of a syndrome of Badda-Kiari
Badda-Kiari's syndrome can be suspected at development of characteristic symptoms: ascites and a gepatomegaliya against the background of increase in coagulability of blood. For final diagnosis the gastroenterologist needs to conduct additional examinations (tool and laboratory).
Laboratory methods yield the following results: the general blood test shows increase in number of leukocytes and SOE; the koagulogramma reveals growth of protrombinovy time, biochemical blood test defines increase in activity of enzymes of a liver.
Tool methods (ultrasonography of a liver, doppler sonography, a portografiya, liver KT, MPT) allow to measure the sizes of a liver and spleen, to define degree of diffusion and vascular disorders, and also a cause of illness, to find blood clots and a stenosis of veins.
Treatment of a syndrome of Badda-Kiari
Medical actions are directed to restoration of a venous blood-groove and elimination of symptoms. Conservative therapy gives temporary effect and promotes minor improvement of the general state. To patients appoint the diuretics bringing excess liquid out of an organism and also the medicines improving a metabolism in cells of a liver. For knocking over of pain and processes of an inflammation use glucocorticosteroids. All patient antiagregant and the fibrinolitik increasing the speed of a rassasyvaniye of blood clot and improving rheological properties of blood write out. The lethality at the isolated use of methods of conservative therapy makes 85-90%.
It is possible to restore blood supply of a liver only in the operational way, but surgical procedures are carried out only in the absence of a liver failure and thrombosis of hepatic veins. As a rule, make the following types of operations: imposing of anastomoz - artificial messages between the damaged vessels; shunting - creation of additional ways of outflow of blood; transplantation of a liver. At a stenosis of the top hollow vein its prosthetics and expansion is shown.
Forecast and complications of a syndrome of Badda-Kiari
The forecast of a disease unfavourable, influences it age of the patient, the accompanying chronic pathology, existence of cirrhosis. Lack of treatment comes to an end with the death of the patient in terms from 3 months up to 3 years after diagnosis. The disease can cause a complication in the form of hepatic encephalopathy, bleedings, a heavy renal failure.
Are especially dangerous sharp and lightning character of a current of a syndrome of Badda-Kiari. Longevity of patients with chronic pathology reaches 10 years. The forecast depends on success of surgery.