Wang Ying,Ma Lishuang,Liu Chao,et al.Characteristics of prenatal diagnosis of congenital lung developmental malformation and timing of surgery during neonatal period[J].Journal of Clinical Pediatric Surgery,2023,22(08):713-718.[doi:10.3760/cma.j.cn101785-202306042-003]
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
- Title:
- Characteristics of prenatal diagnosis of congenital lung developmental malformation and timing of surgery during neonatal period
- Keywords:
- Congenital Lung Malformations; Ultrasonography; Prenatal; Genetic Counseling; Prenatal Diagnosis; Surgical Procedures; Operative
- 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3 135.42 g，最低出生体重2 200 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。
- Abstract:
- 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.
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备注/Memo
收稿日期:2023-06-27。
基金项目: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）
通讯作者:马立霜,Email:malishuang2006@126.com