儿童及青少年布加综合征的介入治疗

摘要

目的 探讨儿童及青少年布加综合征介入治疗的安全性和有效性.方法 1990年12月至2012年8月收治25例布加综合征患儿,男16例,女9例,年龄5～17岁,平均(14.5±3.4)岁.本组均行彩超检查,其中12例行磁共振静脉成像(MRV)检查,诊断为布加综合征.25例均行造影检查,其中下腔静脉型5例、肝静脉型15例、混合型5例.其中24例行介入治疗并术后随访,1例未治疗.结果 24例行介入治疗后受累下腔静脉、肝静脉即刻畅通,肝静脉压力由术前(42.1±4.2)cm H2O(37 ～ 50 cm H2O)(1 cm H2O=0.098 kPa)即刻降至术后(17.3±3.3) cm H2O(14 ～26 cm H2O),下腔静脉压力由术前(30.6+2.9)cm H2O(26～36 cm H2O)即刻降至(18.8±4.2)cm H2O(15 ～26 cm H2O).症状和体征即刻消失,无一例出现并发症,总体初次成功率96％.术后随访6个月～8年,平均25.8个月,7例出现再狭窄,5例给予血管成形术、1例给予血管成形术+置管溶栓+血管成形术、1例给予血管成形术+支架置人治疗后患者受累静脉再次畅通,临床症状明显减轻.结论 介入治疗儿童及青少年布加综合征安全、有效.%Objective Due to its minimal-invasive approach,endovascular procedure had replaced surgery in treating Budd-Chiari syndrome (BCS).The interventional therapy was a safe and effective treatment in adults with BCS and the cure rate was high.However Budd-Chiari syndrome in children and adolescents is rare.Published literature on interventional procedure for Budd-Chiari syndrome in children and adolescents is scarce.The aim of the study was to present results of percutaneous transluminal angioplasty (PTA) and stents placement in children and adolescents with BCS and to evaluate the efficacy and safety in these patients of this approach.Method Twenty-five patients [16 boys and 9 girls; average age of (14.5 + 3.4) years old; age ranged from 5 to 17 years] with Budd-Chiari syndrome who were hospitalized from December 1990 to August 2012 were presented.All of them were diagnosed by color Doppler ultrasound scan while 12 of them had magnetic resonance venography (MRV) scan.All of the patients had undergone angiographic examination.Four cases with membranous obstruction of the inferior vena cava (IVC) were treated with PTA.One case with segmental block of IVC was treated with PTA and stent placement.Five cases with membranous obstruction of IVC and hepatic vein (/and accessory hepatic vein) were treated with PTA.Among 8 cases with membranous obstruction of hepatic veins,6 cases were treated with PTA and the others with PTA and stent placement.Among 4 cases with blocks of 3 hepatic veins (HVs),one was treated with PTA,one with PTA plus catheter thrombolysis plus PTA,one with PTA and stent placement and the other one was unsuccessful.Three cases with obstruction of HV and accessory HV (AHV) were treated with PTA.Totally,24 patients were treated with interventional approach and followed up.Result The procedure was successful in 24 patients.The involved veins (hepatic veins or IVC) were patented after interventional procedure.The pressure of hepatic vein was (42.1 + 4.2) cm H2O (37-50 cm H2O) (1 cm H2O =0.098 kPa) before the interventional therapy,while it was (17.3 + 3.3) cm H2O (14-26 cm H2O) after it.The pressure of IVC was (30.6±2.9) cm H2O (26-36 cm H2O) before the interventional therapy,while it was (18.8 +4.2) cm H2O (15-26 cm H2O) after it.The symptoms and signs vanished instantly after interventional procedure.There were no procedure-related complications.The rate of overall initial cure was 96％.The patients were followed up for a mean of 25.8 months (range 6 months to 8 years).Seven cases developed restenosis after first procedure.Five of them were treated with PTA,one with PTA plus catheter thrombolysis plus PTA,one with PTA and stent placement.All of the involved veins were patented again.Clinical symptoms were relieved.There were no procedure-related complications as well.Conclusion The interventional procedure in children and adolescents with BCS is the same as in adults.Radiological therapeutic intervention is efficacious and safe in children and adolescents with BCS.