Kage M, Arakawa M, Kojiro M, Okuda K
First Department of Pathology, Kurume University School of Medicine, Japan.
Gastroenterology. 1992 Jun;102(6):2081-90. doi: 10.1016/0016-5085(92)90336-w.
It is generally believed that membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome is caused by congenital malformation. However, it does not explain the late onset of the disease. In the current study, hepatic portion of the inferior vena cava and hepatic veins were studied in 17 autopsy cases of the Budd-Chiari syndrome, 16 of which had no demonstrable cause (idiopathic). A sufficient amount of vena cava tissue was available for evaluation in 15 cases. Nine had membranous obstruction, with thickness varying from 3 to 8 mm. Thrombus formation was recognized in 7 of 9 cases. Occlusion of hepatic vein orifices of varying degree was present in 8 cases. In these occluding lesions, the basic structure of the venous wall was maintained. The intima was transformed into a fibrous laminar structure, and organized thrombi of varying ages were recognized; they were a mixture of fresh thrombi, organized thrombi, fibrous tissues, recanalizations, and calcifications. It is concluded that in these cases of the Budd-Chiari syndrome, occluding and stenosing lesions in the inferior vena cava and hepatic veins were thrombosis and its sequelae. There was no indication of congenital malformation.
一般认为，布加综合征中肝下腔静脉膜性梗阻是由先天性畸形引起的。然而，这并不能解释该病的迟发性。在本研究中，对17例布加综合征尸检病例的肝下腔静脉和肝静脉进行了研究，其中16例无明显病因（特发性）。15例有足够的腔静脉组织可供评估。9例有膜性梗阻，厚度为3至8毫米。9例中有7例发现有血栓形成。8例存在不同程度的肝静脉口闭塞。在这些闭塞性病变中，静脉壁的基本结构得以保留。内膜转变为纤维层状结构，可见不同时期的机化血栓；它们是新鲜血栓、机化血栓、纤维组织、再通和钙化的混合物。得出的结论是，在这些布加综合征病例中，肝下腔静脉和肝静脉的闭塞性和狭窄性病变是血栓形成及其后遗症。没有先天性畸形的迹象。
