Abernethy畸形的影像学表现

目的提高对Abemethy畸形（Abemethy malformation）的认识。方法报告2例经DSA证实的罕见Abemethy畸形的影像学表现，2例分别行内科保守治疗和部分性脾动脉栓塞术，并作文献复习。结果例1CT增强扫描及三维血管成像示：肝下段下腔静脉增宽，门静脉干及分支变细，胃和脾周可见迂曲扩张的血管。经肠系膜上动脉间接门静脉DSA可见门静脉干及分支明显变细，肠道静脉血少部分回流入门静脉，大部分经一迂曲扩张的分流道人左。肾静脉；脾动脉DSA示脾静脉血主要经分流道人左。肾静脉。例2CT示：肝叶比例失调，右叶稍大，肝裂宽，脾大，脾门血管迂曲扩张。经肠系膜上动脉间接门静脉DSA可见门静脉干闭塞，周围可见细小迂曲的血管，肠道静脉血大部分经一分流道人左肾静脉；脾动脉DSA显示脾静脉血主要经分流道人左。肾静脉和下腔静脉。结论Abemethy畸形的诊断主要依据影像学，以间接或直接门静脉造影为金标准。治疗方法应根据畸形的不同类型及病人的情况决定。

The imaging manifestations of Abernethy malformation

Objective To enhance the understanding of Abernethy malformation. Methods Two patients (one 9-year-old boy and one 24-year-old girl) with Abernethy malformation confirmed by arterial portography were reported. The imaging findings were described in this report. The 2 patients were treated with medicine and partial embolization of the splenic artery, respectively. The literatures were reviewed. Results The characteristic imaging findings were absent, occluded or narrowed portal vein and a dilated and tortuous extrahepatic portosystemic shunt. Conclusion The diagnosis of Abernethy malformation is based on the imaging features, especially on the direct or arterial portography. The choice of therapeutic method is according to the clinical manifestations and patterns of the Abernethy malformation.