楔入法门静脉造影在儿童门静脉海绵样变中的应用价值

Value of portal wedged venography in pediatric cavernous transformation of portal vein

目的探讨经肝静脉楔入法门静脉造影在儿童门静脉海绵样变（CTPV）中的应用价值与介入操作的可行性。方法2014年9月至2015年10月我院所收治的临床诊断为肝门静脉海绵样变患儿8例，男5例，女3例，年龄1岁2个月～8岁，平均年龄5．6岁，术前Child-Pugh分级8例均为A级；主要临床症状为脾大，呕血，黑便等。8例患儿均行经肝静脉楔入法门静脉造影。结果本组8例患儿中7例楔入法造影显示肝内门静脉走行规则，形态纤细，Rex隐窝与门静脉左、右支及分支显影清晰，该7例患儿均在全身麻醉下行肠系膜上静脉-门静脉左支旁路分流术（Rex术），手术顺利，术后随访3～12个月，影像学检查示吻合口通畅，未见狭窄；另1例患儿造影显示肝内门静脉Rex隐窝及左、右支均未见显影，末端分支显影清晰，术中证实门脉左支闭塞，改行脾肾静脉分流术（War—ren术），本组8例CTPV患儿术前楔入法造影显示门脉左支是否通畅诊断准确率达100％。结论经肝静脉楔入法对CTPV儿童进行门静脉造影能准确诊断患儿门脉左支是否通畅，且能获得良好的肝内门静脉各级分支及Rex隐窝解剖位置及形态大小的图像，对CTPV儿童Rex术前评估意义重大。

Objective To explore the diagnostic value and feasibility of portal wedged venography for pediatric cavernous transformation of portal vein （CTPV）. Methods From September 2014 to October 2015, a total of 8 CTPV children received portal wedged venography. There were 5 boys and 3 girls with a mean age of 5.6 （7/6-8） years. All preoperative liver functions were at level A. The major clinical manifestations included splenomegaly, hematemesis and hematochezia. Results Rex recessus and intrahepatic portal vein with regular and slender shapes were distinctly visualized in 7 patients and all of them underwent mesenteric-to-left portal vein byl=tass （Rex＇s）. There was no imaging evidence of anastomotic stenosis during a follow-up period of 3-12 months. However, it showed only distal branches of intrahepatic portal vein in another child. Rex recessus and left or right branch were poorly visualized. Splenorenal vein shunt （Warren＇s） was performed after confirming occlusion of left branch of intrahepatic portal vein. Conclusions Anatomical location, shape and size of intrahepatic portal vein and Rex recessus of pediatric CTPV are well-delineated on wedged hepatic venous portography. And it is an effective tool of detailed preoperative assessments for identifying the candidates of Meso-Rex bypass.