下腔静脉隔膜阻塞型布-加综合征的病理学及病因学研究

Pathologic and etiologic studies of membrane obstruction of the inferior vena cava in Budd-Chiari syndrome

目的研究下腔静脉隔膜阻塞型布-加综合征(BCS)的解剖位置和隔膜病理特点,以探讨病因及发病机制。方法分析100例非BCS死亡成人解剖资料,进行下腔静脉膈肌以上至右心房的大体观察。常规显微镜病理检查70例下腔静脉隔膜阻塞型患者的隔膜活检材料及20例BCS患者根治术后切除的完整隔膜。结果大体观察发现1例(1%)在位于平膈肌水平距下腔静脉入右心房28mm处、肝静脉开口上方的左外侧壁,存在一瓣膜;47%成人位于下腔静脉近右心房口存在Eustachian瓣。镜检显示,上述全部隔膜符合血管瓣膜样结构。其中30%(21/70)样本附有机化血栓。9%(6/70)样本内有少量炎细胞浸润。全部完整隔膜病理检查与血管壁相延续。结论首次报道在位于下腔静脉平膈肌水平有一瓣膜,这一瓣膜的发现,可能与下腔静脉隔膜阻塞型BCS的发生高度相关。

Objective To study the anatomical location and pathology of membranous obstruction of the inferior vena cava （IVC）in Budd-Chiari syndrome （BCS）with research on the etiologic mechanism and pathology. Methods Analysis of 100 normal adults was performed including the gross anatomy of IVC segment from the level of diaphragm up to right atrium. The conventional, microscopic pathologic examination of the biopsy sampling IVC obstruetice mambrane tissue in 70 cases toghther with the complete resected membrane from the radical therapy for 20 cases of BCS, were collected and under investigation. Results The macroscopic examination revealed the obstructive membrane in one case （1%）localizing at the diaphragmatic level, approximately 28 mm, away from the IVC entrance into the right atrium and a newly found valvula was seen on the left lateral wall of the upper part of the hepatic vein orifice. Simultaneously, 47% adults showed Eustachiun valve existing in IVC near the entrance to right atrium. Microscopy confirmed all the forementioned membranes consisting of vascular valvular structures. Among them （21/70）, 30% showed additional organized thrombus formations, and 9%（6/70）with a few amount of inflammatory cellular infiltrations. The total intact resection membrane was continuous with the vascular wall under microscopic examination. Conclusions The first newly report of the existence of a special valvula at the diaphragmatic level of IVC reveals the possibility of high correlation with the occurance of IVC membranous obstruction type in BCS.