Badda-Chiari Syndrome

Budd-Chiari syndrome occurs due toprogressive obliteration (closing or narrowing) of the lumen of the veins, flowing into the liver. Chiari believed that the main reason - the primary inflammation of the hepatic veins. But later it was revealed in some cases at the same time hepatic thrombosis and inferior vena cava.

The literature describes about 200 cases of obliterating endoflebitis in the hepatic veins (Budd-Chiari disease).

With the development of thrombophlebitis of the arnica duct,which flows directly into the left hepatic vein, and the umbilical vein, this syndrome may develop in early childhood. The inner lining of the hepatic veins or the inferior vena cava (near the confluence of the hepatic veins) expands, sometimes thrombi join. All this causes the closing or narrowing of the lumen of the vein, which, in turn, causes the development of stagnation and compression of the liver cells.

Budd-Chiari syndrome also develops whenother pathologies: malignant neoplasms of the liver, pancreas, kidneys, etc., diseases of generalized blood vessels. Cirrhosis can also contribute to the narrowing of the hepatic veins. They can cause Chiari syndrome with a certain localization of gum, echinococcosis, actinomycosis, abscesses, and so on.

Clinically, Budd-Chiari syndrome manifests itselfsigns of the underlying disease that caused it, in addition, the manifestations also depend on the duration of the blockage of veins. There is an acute and gradual onset of the syndrome.

Acute hepatic vein blockage causesthe sudden appearance of vomiting, pain in the right hypochondrium or epigastria due to swelling of the liver, as well as overdistension of the glisson capsule, ascites develops, a slight yellowness occurs.

At the same time, portal hypertension develops due to a rapid increase in pressure in the hepatic sinusoids and veins.

Ascites with this pathology can not be treateddiuretics, the fluid very quickly accumulates after the paracentesis, sometimes there is blood entering into it, the development of hydrothorax is possible. If a thrombosis joins, which develops in the inferior vena cava, then swelling occurs on the lower extremities, the “jellyfish head”, and with light pressure on it, the blood moves upwards.

With the development of total obstruction of the veins of the liverthrombosis of the mesenteric vessels joins, pains and diarrhea occur, complications of a neurological nature arise due to damage to the liver cells, which are fatal in a few days.

Sometimes Budd syndrome - Chiari, with partialclosure of the lumen of the veins, may occur as a chronic disease for a long time. With this nature of the course, collateral circulation of blood appears over time: the superficial veins of the skin dilate, portal hypertension appears, and then the esophageal veins are also varicose.

Essential for diagnosishas the presence of reduced occlusive pressure in the veins of the liver and a simultaneous increase in portal pressure, which is detected using the methods of hepato- and splenomanometry. Used retrograde X-ray hepatography, during which contrast agents are injected into the hepatic veins. Ultrasound diagnostics is based on direct signs - visualization of a blood clot in the veins.

Without a color Doppler study, Budd-Chiari syndrome can be suggested for:

• The presence of diffuse changes in the liver, characteristic of severe fatty liver or fibrosis of the liver tissue,

• There are no hepatic veins on the image, for example, due to the presence of echopositive masses in the lumen,

• Narrowed view of the inferior vena cava,

• Signs of portal hypertension if the disease is prolonged.