分流血管结扎治疗Abernethy畸形七例

Efficacy of shunt vessel ligation for Abernethy malformation: a report of 7 cases

目的探讨分流血管结扎治疗Abernethy畸形的可行性及效果。方法回顾性分析首都儿科研究所附属儿童医院2014年5月至2017年8月收治并行分流血管结扎手术的7例Abernethy畸形患儿临床治疗过程。全部7例患儿于术前行血管超声及增强CT等检查提示未发现门静脉主干或发现门静脉主干及分支纤细,均表现为肝脏体积小,血氨水平升高。7例患儿中5例为肠系膜下静脉与髂静脉分流,1例为脾静脉与左肾静脉分流,1例为门静脉主干与下腔静脉异常血管分流。术中阻断前通过肠系膜静脉行门静脉测压及造影,完全阻断分流血管后再行测压及造影。若阻断后造影见门静脉主干及肝内门静脉显影,则确诊为Abernethy畸形Ⅱ型,保持阻断状态观察20 min,若无明显异常,且压力低于25 cmH2O(1 cmH2O=0.098kPa),则完全结扎分流血管;若压力高于25 cmH2O,则行分流血管部分结扎,调整并维持压力在25 cmH2O左右,二期手术再行完全结扎分流血管。结果经造影证实全部患儿均为Abernethy畸形Ⅱ型。全部患儿阻断后再次造影均见门静脉较前增宽,肝内门静脉分支显影范围扩大,入肝血流明显增加。3例一期完全结扎,另4例分期结扎,其中2例早期患儿结扎同期施行了Rex术。全部患儿术后肝脏体积均较前明显增加,术后2周内增加了(207.00±54.29)ml,增长了(41.50±13.43)%。结论分流血管结扎治疗Abernethy畸形可行、有效。

Objective To explore the feasibility and outcomes of shunt vessel ligationy for Abernethy malformation (AM). Methods Retrospective analysis was performed for the clinical data of 7 AM children undergoing shunt vessel ligation from May 2014 to August 2017. Preoperative examinations of vascular ultrasound and enhanced computed tomography (CT) failed to detect any slimming of portal vein trunk, portal vein or its branches. Clinical manifestations included small hepatic volume and hyperammonemia. There were inferior mesenteric vein & iliac vein shunt (n=5), splenic vein & left renal vein shunt (n=1) and portal vein trunk & inferior vena cava shunt (n=1). During operation, measuring the pressure of portal vein and contrasting radiography were performed via mesenteric vein prior to blockade. After blockade, the procedure was repeated. If portal vein trunk and its intrahepatic branch were visualized by radiography after blockade, Abernethy malformation typeⅡwas confirmed. During blockade, after 20-minute observations, if there was not any abnormality and the pressure was under 25cmH2O, shunt vessel was ligated completely. If pressure exceeded 25 cmH2O, shunt vessel was partially ligated and pressure was maintained at around 25 cmH2O. During re-operation, shunt vessel was completely ligated. ResultsAM typeⅡ was confirmed by radiography in all cases. After blockade, portal vein trunk and its intrahepatic branch expanded and blood inflow increased significantly. Ligation was one-stage (n=3) and two-stage (n=4). Two early cases underwent a Rex procedure simultaneously. Liver volume expanded markedly post-operation in all cases. At 2 weeks postoperatively, average liver volume increased by 207.00±54.29 ml with a growth rate of 41.50±13.43%. Conclusions Shunt vessel ligation is both feasible and effective for AM.