Budd Chiari Syndrome..

Budd Chiari Syndrome is an uncommon condition caused by narrowing or obstruction of veins of the liver. The syndrome is characterized by hepatomegaly, ascites, and abdominal pain. Any obstruction of the venous vasculature of the liver causes increased portal vein, and hepatic sinusoid pressures as the blood flow stagnate. The increased portal pressure causes increased filtration of vascular fluid with the formation of ascites in the abdomen and collateral venous flow through alternative veins leading to esophageal, gastric, and rectal varices. Obstruction also causes centrilobular necrosis and peripheral lobule fatty change due to ischemia. If this condition persists chronically, a condition called nutmeg liver will develop. Renal failure may occur, perhaps due to the body sensing an “underfill” state and subsequent activation of the renin-angiotensin pathways and excess sodium retention. CAUSES The exact cause of Budd–Chiari syndrome is not known. However, about 10% of individuals with this syndrome have polycythemia vera(a slow-growing blood cancer in which bone marrow makes so many red blood cells). Symptoms develop with blockage of the major veins that carry blood from the liver to the heart; this blockage is usually due to clotting or overgrowth of fibrous tissue in the veins. Other causes include- Primary Budd–Chiari syndrome - thrombosis of the hepatic vein (75%) Secondary Budd–Chiari syndrome - compression of the hepatic vein by an outside structure (e.g., a tumor) (25%) Hepatic vein thrombosis SYMPTOMS Jaundice Enlarged spleen Ankle edema Stasis ulcerations The prominence of collateral veins Abdominal pain Ascites Liver enlargement DIAGNOSIS The diagnosis of Budd–Chiari syndrome is made based on clinical evaluation, a detailed patient history, and various specified tests. Here are a few tests that help diagnose the condition: Ultrasound - may show obliteration of hepatic veins, thrombosis or stenosis, spiderweb vessels, large collateral vessels, or a hyperechoic cord replacing a normal vein CT scan or MRI of the abdomen - show enlarged liver, an irregular pattern of veins in the liver, and other abnormalities Biopsy of the liver tissue TREATMENT Treatment of Budd–Chiari syndrome is highly successful if diagnosed in the early stages. Here are various treatment options: Medications- high doses of corticosteroids, prednisone, anticoagulants such as heparin and warfarin to prevent blood clots, diuretics to control ascites Sodium restriction to control ascites Angioplasty or surgical widening of affected veins may ease high blood pressure in the vessel walls. In some cases, the condition is treated surgically by diverting blood flow from one vein to another, a process called shunting The stunt is also inserted into the vein in some cases to maintain blood flow. Liver transplantation is recommended in severe cases.

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Thanx for illustrative understanding of budd chiari syndrome Sir how the liver transplant will help in portal venous obstructive pathology

Thanx dr Dinesh Gupta
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Very nice illustration sir Budd Chiari syndrome is life threatening condition Need to be diagnosed early and treated early

Very nicely illustrated in a simple way apriciable post thanks for sharing this valuable informative post doctor

Thanks Dr Dinesh Gupta
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Very well enumerated and well explained.useful.

Valuable informative post

BEAUTIFUL ILLUSTRATION

Nice posting Doctor

Informative post sir

Well explained

Good one

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