[A study on the etiology of Budd-Chiari syndrome].

OBJECTIVE

To investigate the etiology of Budd-Chiari syndrome (BCS) with finding in surgical operation.

METHODS

Thirteen patients with BCS undertook direct vision membranectomy and reconstruction of inferior vena cava. The specimens were studied with light microscopy, electron microscope and immunohistochemistry.

RESULTS

Membranous occluding changes, which thickness varying from 2 to 20mm, were found in the posthepatic segment of inferior vena cava during the operation. Thrombi in inferior vena cava were found in 7 cases, 3 of them with thrombi evenly extending to the root of hepatic vein. The compromised venous wall showed proliferation of fibrous connective tissue, hyalinization or mucoid degeneration under light microscopy, 9 of the 13 cases showed chronic infiltration of inflammatory cells. Copious collagenous fibers, few endothelial cells and mitochondria swelling were found with electron microscope. No immune complex (IgA, IgG, IgM) was observed by immunohistochemistry.

CONCLUSION

It is concluded that in these cases of BCS, occluding and stenosing lesions in the inferior vena cava were the outcome of thrombosis and its sequelae and local inflammation might be one of the reasons of thrombosis. There was no evidence of congenital malformation.