Management of Idiopathic Budd-Chiari Syndrome with Primary Stent Placement: Early Results

Abstract

To evaluate the utility of primary stent placement in the management of Budd-Chiari syndrome (BCS) secondary to idiopathic inferior vena caval (IVC) obstruction. The case records of nine patients (four women, five men), ranging in age from 22 to 58 years (median, 26 years), with idiopathic IVC obstruction were reviewed. Hepatosplenomegaly, esophageal varices, and prominent collateral veins were found in all patients, while four also had ascites. Hepatic functional reserve was graded as Child class A in three patients and class B in the remaining six. All had at least one patent hepatic vein opening into the IVC below the site of occlusion. Percutaneous angioplasty of the IVC was performed, followed by the placement of double-skirt Gianturco-Rösch or hybrid Gianturco stents. Clinical follow-up was supplemented with duplex ultrasound (n = 8), endoscopy (n = 4), and cavography (n = 2). Caval lesions were segmental. Revascularization was technically successful in all patients. The median pressure gradient across the lesion dropped from 38 mm Hg (range, 27-61 mm Hg) to 15 mm Hg (range, 10-20 mm Hg) (P = .008). Residual stenosis after stent placement ranged from 9% to 40% (median, 20%). One patient died of presumed pulmonary embolism; another patient experienced an episode of epistaxis. The procedure was followed by regression of signs and symptoms in the eight survivors. During the follow-up period (range, 3-31 months; median, 7 months) the IVC remained patent in all patients, and clinical features of BCS did not recur. Primary stent placement could serve as the first line of treatment in patients with idiopathic BCS when the underlying lesion is not amenable to angioplasty.