永存第五主动脉弓缩窄和第四主动脉弓中断的外科治疗  

Surgical repair of persistentⅤaortic arch coarctation and interruptedⅣaortic arch

作　　者：施旭聪 翁建彬 范铭 濮乙青 俞劲[1] 应力阳[1] 石卓[1] 范祥明 Shi Xucong;Weng Jianbin;Fan Ming;Pu Yiqing;Yu Jin;Ying Liyang;Shi Zhuo;Fan Xiangming(Department of Cardiac Surgery,Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310052,China;Department of Neurosurgery,Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310052,China;Department of Ultrasound Diagnosis,Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310052,China;Operation Room,Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310052,China)

机构地区：[1]浙江大学医学院附属儿童医院心脏外科,国家儿童健康与疾病临床医学研究中心,杭州310052 [2]浙江大学医学院附属儿童医院神经外科,国家儿童健康与疾病临床医学研究中心,杭州310052 [3]浙江大学医学院附属儿童医院超声科,国家儿童健康与疾病临床医学研究中心,杭州310052 [4]浙江大学医学院附属儿童医院手术室,国家儿童健康与疾病临床医学研究中心,杭州310052

出　　处：《中华小儿外科杂志》2023年第12期1071-1075,共5页Chinese Journal of Pediatric Surgery

摘　　要：目的探讨永存第五主动脉弓(persistent fifth aortic arch,PFAA)的解剖特征及手术方式,以期改善患儿的预后和更好的指导临床工作。方法收集2018年1月至2022年12月浙江大学医学院附属儿童医院诊断和治疗的5例体-体型PFAA患儿的临床资料,其中男2例,女3例;均在新生儿期经计算机断层血管造影明确诊断,其中4例是第五主动脉弓缩窄合并第四主动脉弓中断,另1例是主动脉双腔无狭窄,均无严重的心内畸形。根据患儿的临床特征、影像数据和手术资料,总结分析其解剖特征及诊断鉴别点,讨论手术矫治方法及术后随访结果。结果2例患儿采用了两种不同的手术方法矫治,其中1例术后随访至今4年余无狭窄,而另1例术后6个月出现再狭窄给予经皮球囊扩张治疗。1例进行染色体检查后发现异常,家属放弃治疗死亡。1例合并复杂的主动脉及其分支变异:右侧PFAA合并右侧第四主动脉弓中断,伴迷走左锁骨下动脉及左弓残端Kommerell憩室。结论计算机断层血管造影是明确诊断PFAA最有效的无创检查方法。手术矫治应尽可能完整地切除第五主动脉弓的组织来防止术后再狭窄。Objective To summarize the management experiences and follow-up outcomes of persistent fifth aortic arch(PFAA)cases and to explore their anatomical characteristics and surgical approaches for optimal outcomes.Methods From January 2017 to December 2022,retrospective review was performed for 5 children hospitalized with systemic-systemic type PFAA.There were 2 boys and 3 girls.A definite diagnosis was made by computed tomography angiography(CTA)in neonatal period.There were coarctation of the fifth aortic arch with interruption of the fourth aortic arch(n=4)and double-lumen aortic arch without stenosis(n=1).None had serious intracardiac malformation.Clinical features,imaging studies and surgical approaches were examined.Results Two cases underwent two different kinds of surgical methods to reconstruct aorta.One case was followed up for over 4 years without stenosis and another case of restenosis at 6 months postoperatively underwent percutaneous balloon dilatation.One girl of chromosomal abnormality died after declining treatments.One case complicated with complex variation of aorta and its branches became lost during follow-ups.The anatomical combination of right PFAA with interruption of right fourth aortic arch,aberrant left subclavian artery and Kommerell diverticulum at the stump of left arch has not been reported in the literature.Conclusions CTA is the most effective non-invasive diagnostic tool for PFAA.In light of the histological characteristics of PFAA,tissue of fifth arch should be removed as completely as possible to prevent postoperative restenosis.

关 键 词：先天畸形 永存第五主动脉弓 主动脉缩窄 主动脉弓中断 手术治疗 

分 类 号：R726.5[医药卫生—儿科]