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Badda-Chiari Syndrome: Causes, Symptoms, Diagnosis, Treatment
Last reviewed: 23.04.2024
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Badda-Chiari syndrome is an occlusion with a violation of venous outflow from the hepatic veins, which can be localized at the level from the right atrium to small branches of the hepatic veins. The manifestation of this lesion varies from asymptomatic to fulminant liver failure. The diagnosis is based on ultrasound. The treatment of Badda-Chiari syndrome includes symptomatic drug therapy and is aimed at restoring the permeability of veins through thrombolysis, decompression shunting and long-term anticoagulant therapy.
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What causes the Budda-Chiari syndrome?
Occlusion of small branches of hepatic veins is combined with occlusive diseases of veins. In Western countries, the main cause of the Badd-Chiari syndrome is thrombosis of the hepatic vein into the lower vena cava. The main etiological factors include thrombotic coagulopathy (eg, deficiency of protein C or S, antithrombin III, pregnancy, use of oral contraceptives), hematological diseases (eg polycythemia, paroxysmal nocturnal hemoglobinopathy, myeloproliferative disorders), inflammatory bowel disease, connective tissue diseases and trauma . Other causes include infections (eg, hydatid cysts, amoebiasis) and tumor germination into the hepatic veins (eg, hepatocellular carcinoma or renal cell carcinoma). The causes of venous obstruction are often unknown. In Asia and South Africa, the cause is often the membranous obstruction of the inferior vena cava above the liver, probably due to the recanalization of a thrombus formed in adulthood, or the developmental defect in childhood.
Consequences of venous occlusion are ascites, portal hypertension and hypersplenism.
Symptoms of Badd-Chiari Syndrome
The manifestation of the syndrome varies from asymptomatic to fulminant development of hepatic insufficiency or cirrhosis of the liver. Acute occlusion (classical Badd-Chiari syndrome) is manifested by pain in the right upper quadrant of the abdomen, nausea, vomiting, mild jaundice, enlarged and painful liver, ascites. With complete occlusion of the inferior vena cava, swelling of the abdominal wall and lower extremities develops with a pronounced expansion of the superficial veins of the abdomen from the pelvis to the costal arch. Subacute course (<6 months) is manifested by hepatomegaly, coagulopathy, ascites, splenomegaly, bleeding from varicose veins and hepatorenal syndrome. In most cases, there is a chronic process (> 6 months), accompanied by fatigue, the presence of convoluted superficial veins of the abdomen, in some patients bleeding from varicose nodules, ascites and decompensated cirrhosis of the liver.
Where does it hurt?
Diagnosis of Budd Chiari Syndrome
Suspicion of the Budda-Chiari syndrome occurs with the development of hepatomegaly, ascites, liver failure, or liver cirrhosis, and a combination of abnormalities in functional hepatic tests with risk factors for thrombosis. The results of Doppler ultrasound show blood flow disturbances and occlusion sites. Indications for CT and MRI are the noninformativity of ultrasound. If surgery is planned, angiography is necessary. Laboratory tests are not diagnostic, but help in assessing the functional state of the liver.
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The prognosis and treatment of the Budd Chiari syndrome
In most cases, patients with complete occlusion of veins die from liver failure for 3 years. With incomplete occlusion, the course of the disease is variable.
Treatment for Badd-Chiari syndrome includes symptomatic therapy of complications (eg, ascites, liver failure) and decompression. Thrombolysis allows lysing acute blood clots and reducing hepatic congestion. With membranous obstruction of the vena cava or stenosis of the hepatic veins, the outflow is restored by percutaneous transluminal balloon angioplasty with intraluminal stenting. Intrahepatic transureular stenting and many surgical methods of shunting also provide decompression. In the case of encephalopathy, shunting is generally not applied due to the progression of liver failure and impairment of the synthetic function of the liver. In addition, bypass surgery is associated with a risk of thrombosis, especially in hematologic disorders. To prevent relapse, long-term anticoagulant therapy is often necessary. To save the life of a patient with fulminant forms of the disease or decompensated liver cirrhosis, liver transplantation can be performed.