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,,22():713-718.[doi:10.3760/cma.j.cn101785-202306042-003]
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
- 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.
References:
[1] Stocker LJ, Wellesley DG, Stanton MP, et al.The increasing incidence of foetal echogenic congenital lung malformations:an observational study[J].Prenat Diagn, 2015, 35(2):148-153.DOI:10.1002/pd.4507.
[2] Kunisaki SM, Saito JM, Fallat ME, et al.Development of a multi-institutional registry for children with operative congenital lung malformations[J].J Pediatr Surg, 2020, 55(7):1313-1318.DOI:10.1016/j.jpedsurg.2019.01.058.
[3] 中华医学会小儿外科学分会普胸外科学组, 中国医疗保健国际交流促进会妇儿医疗保健分会.先天性肺气道畸形诊疗中国专家共识(2021版)[J].中华小儿外科杂志, 2021, 42(8):679-687.DOI:10.3760/cma.j.cn421158-20210112-00021. Section of General Thoracic Surgery, Branch of Pediatric Surgery, Chinese Medical Association, Branch of Women and Children’s Medical Care, China International Exchange and Promotion Association for Medical and Health Care:National Expert Consensus on Diagnosing & Treating Congenital Pulmonary Airway Malformations in China (Edition 2021)[J].Chin J Pediatr Surg, 2021, 42(8):679-687.DOI:10.3760/cma.j.cn421158-20210112-00021.
[4] Morini F, Zani A, Conforti A, et al.Current management of congenital pulmonary airway malformations:a "European pediatric surgeons’ association" survey[J].Eur J Pediatr Surg, 2018, 28(1):1-5.DOI:10.1055/s-0037-1604020.
[5] Shulman R, Sparks TN, Gosnell K, et al.Fetal congenital pulmonary airway malformation:the role of an objective measurement of cardiomediastinal shift[J].Am J Perinatol, 2019, 36(3):225-232.DOI:10.1055/s-0038-1669909.
[6] Stoiber B, Moehrlen U, Kurmanavicius J, et al.Congenital lung lesion:prenatal course, therapy and predictors of perinatal outcome[J].Ultraschall Med, 2017, 38(2):158-165.DOI:10.1055/s-0035-1553261.
[7] Kunisaki SM, Saito JM, Fallat ME, et al.Fetal risk stratification and outcomes in children with prenatally diagnosed lung malformations:results from a multi-institutional research collaborative[J].Ann Surg, 2022, 276(5):e622-e630.DOI:10.1097/SLA.0000000000004566.
[8] Peters NCJ, Hijkoop A, Hermelijn SM, et al.Prediction of postnatal outcome in fetuses with congenital lung malformation:2-year follow-up study[J].Ultrasound Obstet Gynecol, 2021, 58(3):428-438.DOI:10.1002/uog.23542.
[9] Hegde BN, Tsao K, Hirose S.Management of congenital lung malformations[J].Clin Perinatol, 2022, 49(4):907-926.DOI:10.1016/j.clp.2022.08.003.
[10] Aziz KB, Jelin AC, Keiser AM, et al.Obstetrician patterns of steroid administration for the prenatal management of congenital pulmonary airway malformations[J].J Neonatal Perinatal Med, 2021, 14(2):213-222.DOI:10.3233/NPM-200526.
[11] Griggs C, Schmaedick M, Gerall C, et al.Vanishing congenital lung malformations:what is the incidence of true regression?[J].J Neonatal Perinatal Med, 2022, 15(1):105-111.DOI:10.3233/NPM-210740.
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