先天性门静脉缺如伴肝内多发结节并文献复习

Congenital absence of portal vein with intrahepatic multiple nodules: case report and review of literature

目的:探讨先天性门静脉缺如(congenital absence of portal vein,CAPV)的临床特点、影像学表现及预后。方法:收集1例CAPV患者的临床及影像学资料并复习相关文献。结果:通过肝彩色多普勒超声(color Doppler flow imaging,CDFI)及腹部CT增强扫描(abdominal CT scan,CTA)测得患者肝内巨大富血流占位性病变,肝门静脉系统血流信号缺如,并可见肠系膜上静脉与脾静脉汇合为一宽大下行血管经双侧卵巢静脉汇入双侧髂内静脉。结论:通过CDFI及CTA等检查手段基本可确诊CAPV,回顾相关文献,提高了对该疾病发病机制、病理生理及诊疗预后的认识。

To study the dinical characteristics, imaging findings and prognosis of congenital absence of portal vein Methods： The imaging data of one case of CAPV was organized. And then we learned its pathogenesis, diagnosis and treatment combined with literature. Results： We measured a huge hepatic space occupying lesion enriched in blood, the absence of the blood flow of hepatic portal system and a broad downward vessel mingled in bilateral ovarian veins on their way to bilateral iliac veins confluence with superior mesenteric vein and splenic vein by the way of color Doppler ultrasound （CDFI） and abdominal CT scan （CTA）. Conclusion： CAPV can be diagnosed by means of CDFI and CTA. We improved the awareness of the pathogenesis, pathophysiology, diagnosis and treatment of the disease by reviewing the relevant literature.