Abernethy畸形合并肝脏局灶性结节增生临床治疗经验

Experiences in clinical management of Abernethy malformation combined with focal nodular hyperplasia

目的总结Abernethy畸形合并肝脏局灶性结节增生(FNH)的临床治疗经验。方法以2010年1月至2018年12月解放军总医院第一医学中心肝胆外科收治的3例Abernethy畸形合并FNH患者临床资料为研究对象,其中男性1例,35岁;女性2例,分别为21岁与3岁9个月。患者行数字减影血管造影(DSA)球囊扩张、分流血管结扎术、门静脉(PV)测压等,分析治疗前后PV压力变化。结果DSA提示患者先天性门体分流分别为Abernethy畸形Ⅱ型和Ⅰb型,术前均合并FNH。其中1例5年前因FNH行肝部分切除术,再发腹痛症状入院。3例患者经门腔分流血管缩窄术/门静脉结扎术、脾动脉结扎术/门体分流结扎术处理后PV压力分别由8.5、9.0、20.0 cmH2O(1 cmH2O=0.098 kPa)升至15.0、21.0、25.0 cmH2O,入肝PV血流明显增多,术后DSA造影提示分流血管闭塞,随访至2019年11月均生存良好。结论门腔(体)分流血管结扎或缩窄术可显著改善Abernethy畸形合并FNH患者PV入肝血流,对远期生存获益。

Objective To summarize clinical experience in the management of Abernethy malformation combined with focal nodular hyperplasia(FNH).Methods Three patients with Abernethy malformation combined with FNH who were managed in the Department of Hepatobiliary Surgery,the First Medical Center of Chinese PLA General Hospital from January 2010 to December 2018 entered into this study.There were one male aged 35 years and two females aged 21 years and three years and nine months,respectively.Patients underwent digital subtraction angiography(DSA)with balloon dilation,shunt vasulature ligation,portal vein(PV)pressure measurement and other investigations were performed to study the changes of PV pressure before and after treatment.Results DSA indicated that the congenital portal shunt of the patients was Abernethy deformityⅡandⅠb,respectively,and with combined FNH diagnosed preoperatively.One patient was admitted to hospital for partial hepatectomy 5 years ago for the treatment of FNH,which presented with recurrent abdominal pain.The PV pressure increased from 8.5,9.0,20.0 cmH2O(1 cmH2O=0.098 kPa)to 15.0,21.0,25.0 cmH2O after portal cavity shunt vasculature/portal vein ligation and splenic artery ligation/portal body shunt ligation,respectively.The PV blood flow into the liver increased,and DSA suggested shunt vessel occlusion.On follow-up,all patients survived well.Conclusion Ligation or constriction of shunt vessels significantly improved the flow of PV into liver in Abernethy malformation patients with FNH.The treatment benefited long-term survival.