Badda-Chiari Syndrome

Badda-Chiari syndrome arises from the progressive obliteration (closing or narrowing) of the lumen of veins flowing into the liver. Chiari believed that the main reason - the primary inflammation of the hepatic veins. But in the following cases, thrombosis of the hepatic and inferior vena cava was detected in some cases.

The literature describes approximately 200 cases of occlusive endotheliitis in the hepatic veins ( Badd-Chiari disease ).

With the development of thrombophlebitis of the Aranzian duct, which flows directly into the left hepatic vein, and the umbilical vein, this syndrome can develop already in early childhood. The inner membrane of the hepatic veins or the lower hollow vein (not far from the site of the confluence of the hepatic veins) proliferates, sometimes thrombi attach. All this causes the closure or narrowing of the lumen of the vein, which, in turn, causes the development of stagnation and compression of the liver cells.

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

Clinically, the Buddha-Chiari syndrome manifests itself as a symptom of the underlying disease, which caused it, in addition, the manifestations also depend on the duration of the blockage of the veins. A sharp and gradual onset of the syndrome is indicated.

Acute blockage of the hepatic veins causes a sudden appearance of vomiting, pain in the right hypochondrium or epigastrium due to swelling of the liver, as well as overgrowth of the glisson capsule, ascites develop, a slight icterus appears.

Portal hypertension develops due to the rapid increase in pressure in the hepatic sinusoids and veins.

Ascites in this pathology can not be treated with diuretics, the fluid very quickly re-accumulates after the paracentesis, sometimes the ingestion of blood into it, possibly the development of hydrothorax. If thrombosis develops in the inferior vena cava, swelling on the lower limbs, "jellyfish head", and with a slight pressure on it, blood moves upward.

With the development of total blockage of the veins of the liver, thrombosis of the mesenteric vessels joins, pain and diarrhea occur, complications of a neurological nature appear due to damage to the liver cells, which result in death in a few days.

Sometimes the Badd-Chiari syndrome, with partial closure of the veins, can occur as a chronic disease for a long time. With this nature of the flow, collateral circulation occurs over time: the superficial veins of the skin expand, portal hypertension appears, and then the varicose veins of the esophagus enter.

To make the diagnosis, the presence of a reduced occlusive pressure in the veins of the liver and a simultaneous increase in portal pressure are essential, as revealed by the methods of hepato- and splenomanometry. Retrograde X-ray-hepatopathography is used, during which contrast substances are injected into the hepatic veins. UZ-diagnosis is based on direct signs - visualization of a blood clot in the veins.

Without a color Doppler study, one can assume the Budda-Chiari syndrome with:

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

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

• A narrowed lower vena cava,

• Signs of portal hypertension if the disease persists for a long time.