Budd-Chiari Syndrome

Budd-Chiari syndrome

Budd-Chiari syndrome is an obstruction to the hepatic venous outflow. Two types have been described: (1) Obstruction to the hepatic veins draining into the inferior vena cava (2) Obstruction to the intrahepatic portion of the inferior vena cava, thereby obstructing the hepatic venous outflow. The original descriptions were by Budd in 1845 and Chiari in 1899. Thrombotic obstruction can occur due to thrombotic disorders which may include clotting disorders and myeloproliferative disorders. Chronic inflammatory diseases, intravascular webs and malignancies can also cause obstruction to the hepatic venous outflow. The final result is portal hypertension and hepatic insufficiency.

The clinical presentation  of Budd-Chiari syndrome is with abdominal pain, ascites and hepatomegaly. Splenomegaly and jaundice may also be associated. Peripheral edema and presence of collateral veins over the abdominal wall in case of inferior vena caval obstruction are characteristic features. Collaterals are identified over the anterior abdominal wall with upward flow. In case of cirrhosis liver without hepatic venous obstruction, the collateral flow will be towards the umbilicus. Sometimes lower limb ulcers due to extreme venous stasis can also be seen.

The obstruction can be localized by an ultrasound evaluation of the abdomen, which will also identify tumors causing extrinsic obstruction of the inferior vena cava or the hepatic veins. A venous angiogram, inferior vena cavogram, is needed prior to interventional procedures. Surgical anastomosis has a high risk and hence is often not resorted to. A dual injection angiogram with one catheter above the obstruction, introduced through the jugular route and another catheter below the obstruction introduced through the femoral route will be good to localise the obstruction and the exact extent of the lesion.

Most cases respond well to percutaneous venoplasty with our without stenting. Since there is a chance of thrombus being present in a chronic venous obstruction, thrombolytic therapy prior to intervention has been tried and reported to produce good results. In the earlier era, massive pulmonary thromboembolism have been reported after opening up the chronically obstructed inferior vena cava. Another problem during intervention is to get access to the true femoral vein. Presence of large number of dilated collaterals in the groin region often leads to inadvertent access to one of these. Hence care and repeated attempts are often needed to enter the true femoral vein and then into the inferior vena cava. Collaterals often lead to the hemi-azygous venous system.

Total obstructions can be crossed with the stiff end of the guide wire if usual guide wire access is not possible. Another technique which has been used by some workers is the puncture of the obstruction using transseptal puncture needle. This has to be done by an experienced operator with fluoroscopic imaging in multiple planes, guided by dye injection from above and below. Once the guide wire has been passed, initial dilatation with septal dilator is useful. Mitral valvotomy balloon has also been used for dilating the inferior vena cava. This has been mentioned to be useful in case of tough fibrotic lesions not responding to conventional peripheral balloon dilatations, especially when the tough lesion causes a rupture of the balloon. Care has to taken not to overdilate and produce a rupture of the inferior vena cava which can be catastrophic. Dilatation pressure has to be monitored and the balloon volume adjusted to the potential inferior vena caval diameter, with adequate undersizing for safety.

Persons with gross edema respond with remarkable diuresis after the procedure and have to be monitored for electrolyte imbalance. Hepatic function improves gradually, except in an occasional case with irreversible damage.