Surgical repair in children with the Budd-Chiari syndrome.

Membranous obstruction of the inferior vena cava at the level of the diaphragm is a rare cause of Budd-Chiari syndrome in children. Medical therapy usually fails. Surgical intervention aims at reestablishing patency of the inferior vena cava and hepatic venous outflow to the right atrium. We report on the management of this condition in 19 children of whom 7 were treated surgically. Indications for operation were persistent ascites, deteriorating liver function, and hepatic and inferior vena caval obstruction without significant collateral circulation. Three pathologic types were identified by ultrasonography and cavography and were confirmed at operation. These were type I (4 cases), with a thin membrane occluding the inferior vena cava at the level of the diaphragm; type II (12 cases), with segmental fibrotic obstruction of the inferior vena cava with variable involvement of hepatic veins; and type III (3 cases), with complete absence or nonvisualization of the inferior vena cava. All procedures were done with an extended midline sternotomy incision, cardiopulmonary bypass, core cooling to 16 degrees to 20 degrees C, and periods of circulatory arrest. Type I lesions necessitated membranectomy; type II lesions necessitated transcaval resection of the occluded confluence of the inferior vena cava and the hepatic vein with repair of the defect with an autogenous pericardial patch. One type II lesion, in addition, called for use of a 14 cm polytetrafluoroethylene tube graft to restore inferior vena caval flow. After the operation, marked clinical improvement was observed with an immediate reduction in liver and spleen size and resolution of ascites. Repeat cavography 10 to 30 days after the operation revealed complete patency in four cases and residual stenosis, which required transiliac balloon angioplasty to normalize the inferior vena cava/right atrial pressure gradient, in 3 cases. Thus eventual relief of hepatic venous outflow obstruction and inferior vena caval flow was restored in all cases. We advocate transcardiac membranectomy and pericardial patch grafting for symptomatic and deteriorating membranous obstruction of the inferior vena cava in children.