先天性肺发育畸形产前诊断特点及新生儿期手术时机探讨  

Characteristics of prenatal diagnosis of congenital lung developmental malformation and timing of surgery during neonatal period

作　　者：王莹[1] 马立霜[1] 刘超 李景娜[1] 魏延栋[1] 张艳霞[1] 吴涛[1] Wang Ying;Ma Lishuang;Liu Chao;Li Jingna;Wei Yandong;Zhang Yanxia;Wu Tao(Department of Neonatal Surgery,Children’s Hospital,Capital Institute of Pediatrics,Beijing 100020,China)

机构地区：[1]首都儿科研究所附属儿童医院新生儿外科,北京100020

出　　处：《临床小儿外科杂志》2023年第8期713-718,共6页Journal of Clinical Pediatric Surgery

基　　金：北京市卫生健康委员会高层次公共卫生技术人才建设项目培养计划(01-034)

摘　　要：目的探讨先天性肺发育畸形(congenital lung malformations,CLMs)的产前诊断特点及新生儿期手术指征。方法回顾性分析2019年1月至2022年12月首都儿科研究所附属儿童医院新生儿外科于新生儿期实施手术的12例先天性肺发育畸形患儿临床资料。其中男10例,女2例;足月儿9例,早产儿3例,最小胎龄32^(+5)周;平均出生体重3135.42 g,最低出生体重2200 g。收集所有患儿产前超声检查资料、孕期干预方式、出生后临床特点、手术方式以及病理检查结果。结果12例均获得产前诊断并接受产前咨询。产前诊断为先天性肺气道畸形(congenital pulmonary airway malformation,CPAM)9例,支气管隔离肺(broncho-pulmonary sequestration,BPS)2例,先天性肺叶气肿(congenital lobar emphysema,CLE)1例。产前诊断时间:11例为孕中期(孕20~24周),1例为孕晚期(孕37周)。产前超声动态监测先天性肺气道畸形体积比(congenital pulmonary airway malformation-volume ratio,CVR)最大值为0.68~5.00,末次超声CVR均值1.48,合并不同程度胸/腹腔积液8例,羊水过多3例。12例分娩后均出现不同程度呼吸困难,9例需呼吸支持。术前诊断CPAM 6例,BPS 4例,CLE 2例;术前合并新生儿呼吸窘迫综合征1例,持续肺动脉高压2例,患侧张力性气胸2例。行胸腔镜微创手术6例,开胸手术6例。术后出现气胸1例,经保守治疗痊愈;漏斗胸1例,于3岁时手术矫治,其余患儿恢复良好。结论先天性肺发育畸形产前CVR数值高,分娩后较早出现呼吸困难,需新生儿期手术可能性大。新生儿期密切监测患儿生命体征、呼吸状态,选择恰当的手术时机,可以获得良好预后。Objective To explore the characteristics of prenatal diagnosis and neonatal surgery in children with severe congenital lung developmental malformations.Methods There were 10 boys and 2 girls.And they were term(n=9)and premature(n=3)with a minimal gestational age of 32^(+5)weeks,a mean birth weight of 3135.42 gram and a minimal birth weight of 2200 gram.Prenatal ultrasound examination,pregnancy interventios,postnatal clinical characteristics,surgical approaches and pathological results were recorded.Results All of them obtained a prenatal diagnosis with prenatal consultations.The maximal CVR of dynamic monitoring of prenatal ultrasound was 0.68-5,the mean congenital pulmonary airway malformation-volume(CVR)of the last ultrasound was 1.48,8 cases of thoracic/abdominal effusion of different degrees and 3 cases of excessive amniotic fluid.Delivery was transferred by transport team through a green channel.There were varying degrees of dyspnea after delivery and 9 cases required respiratory support.The preoperative diagnosis was congenital pulmonary airway malformation(CPAM,n=6),broncho-pulmonary sequestration(BPS,n=4)and congenital lobar emphysema(CLE,n=2).There were neonatal respiratory distress syndrome(n=1),persistent pulmonary hypertension(n=2)and unilateral tension pneumothorax(n=2).Thoracoscopic mini-invasive surgery(n=6)and thoracotomy(n=6)were performed.One case of postoperative pneumothorax was cured by conservative measures.Another case of pectus excavatum was surgically corrected at an age of 3 years while the remainders recovered well.Conclusion High antenatal CVR for congenital lung developmental malformation and early dyspnea after delivery hint at a high possibility of surgery during neonatal period.Closely monitoring vital signs and respiratory status and choosing appropriate surgical timing may yield a decent prognosis.

关 键 词：先天性肺发育畸形 超声检查 产前 遗传咨询 产前诊断 外科手术 

分 类 号：R726.5[医药卫生—儿科]