本研究旨在探讨手术结扎术治疗儿童先天性肝外门体分流术(Abernethy畸形)的可行性和有效性。
12名患有Abernethy畸形的儿童(10天至13.3岁;6名男孩和6名女孩)被首都儿科研究所(北京,中国)2014年5月至2019年11月因高氨血症。其中,九人患有便血,两个人肝脏转氨酶水平升高或黄疸,其中一人有低氧血症.回顾性分析其手术方式和结果。在手术期间门体分流术闭塞前后进行门静脉压力测量和门静脉血管造影。六名患者接受了门体分流术的单阶段结扎,5人接受了两期门体分流术结扎,其中一人接受门体分流部分结扎治疗。
每个手术都成功完成,手术时间为60至240分钟,术中失血量为50至200mL。三个孩子输血。所有患儿术后门静脉压力均明显高于术前。随访5~70个月。9例患者术后便血症状缓解。2例患者术后血胆红素和转氨酶水平恢复正常。一名患者的氧饱和度恢复正常。所有患者术后血氨水平均正常。
门体分流术的手术结扎是治疗II型Abernethy畸形的有效方法。根据门体分流闭塞后的门静脉压力,选择了单一或分阶段的程序。
This study aimed to investigate the feasibility and effectiveness of surgical ligation in the treatment of a congenital extrahepatic portosystemic shunt (Abernethy malformation) in children.
Twelve children (aged 10 days to 13.3 years; six boys and six girls) with Abernethy malformation were admitted to the Capital Institute of Pediatrics (Beijing,
China) from May 2014 to November 2019 owing to hyperammonemia. Among them, nine suffered from hematochezia, two had elevated liver transaminase levels or jaundice, and one had hypoxemia. Their surgical procedures and outcomes were retrospectively analyzed. Portal pressure measurement and angiography of the portal vein were performed before and after the occlusion of the portosystemic shunt during the surgery. Six patients underwent a single-stage ligation of the portosystemic shunt, five underwent a two-stage ligation of the portosystemic shunt, and one was treated with a partial ligation of the portosystemic shunt.
Each of the operations was successfully completed with an operative time ranging from 60 to 240 minutes and with an intraoperative blood loss ranging from 50 to 200 mL. Three children had a blood transfusion. The postoperative portal pressure was significantly higher than that before the surgery in all children. The patients were followed for 5 to 70 months. The symptoms of hematochezia were relieved in nine patients after the surgery. The levels of blood bilirubin and transaminase returned to normal after the surgery in two patients. The oxygen saturation returned to normal in one patient. The level of blood ammonia was normal after the surgery in all patients.
The surgical ligation of the portosystemic shunt was an effective method to treat a type II Abernethy malformation. According to the portal pressure after the occlusion of the portosystemic shunt, a single or staged procedure was selected.