Zhao Yunlong,Ma Lishuang,Jiang Yulin,et al.Clinical experience of ex-utero intrapartum treatment and prognostic factors of neonatal diaphragmatic hernia[J].Journal of Clinical Pediatric Surgery,,20():808-813.[doi:10.12260/lcxewkzz.2021.09.002]
Click Copy

Clinical experience of ex-utero intrapartum treatment and prognostic factors of neonatal diaphragmatic hernia

Journal of Clinical Pediatric Surgery[ISSN:1671-6353/CN:43-1380/R] volume: 第20卷 Number: 2021 09 Page: 808-813 Column: 专题·先天性膈疝诊疗新进展 Date of publication: 2021-09-25
Author(s):
Zhao Yunlong1;  Ma Lishuang1;  Jiang Yulin2;  Wang Ying3;  Liu Chao3;  Wei Yandong3;  Li Jingna3;  Lü Yan2;  Jian Shan4;  Meng Hua5;  OuYang Yunshu5;  Feng feng6;  You Hui6;  Pei Lijian7
1. Department of Neonatal Surgery, Peking Union Medical College, Chinese Academy of Medical Sciences, Capital Institute of Pediatrics, Affiliated Children’s Hospital, Beijing 100020, China;
2. Department of Obstetrics, Peking Union Medical College Hospital;
3. Department of Neonatal Surgery, Capital Institute of Pediatrics, Affiliated Children’s Hospital, Beijing;
4. Department of Pediatrics, Peking Union Medical College Hospital, Beijing;
5. Department of Ultrasonography, Peking Union Medical College Hospital, Beijing;
6. Department of Radiology, Peking Union Medical College Hospital, Beijing;
7. Department of Anesthesiology, Peking Union Medical College Hospital, Beijing
Keywords:
Hernias;  Diaphragmatic;  Congenital/SU; Hernias;  Diaphragmatic;  Congenital/DI; Ex-utero Intrapartum Treatment; Treatment Outcome; Comp Study; Infant;  Newborn
CLC:
R655.6;R722.1
DOI:
10.12260/lcxewkzz.2021.09.002
Abstract:
Objective To conduct a retrospective review of our hospital’s single-center experience in the treatment of neonatal congenital diaphragmatic hernia (CDH) and elucidate the relevant prognostic factors.Methods From January 1, 2011 to December 31, 2020, a retrospective review was performed for 95 CDH neonates.All of them received prenatal-perinatal-postpartum multidisciplinary diagnosis and treatments including prenatal screening, prenatal diagnosis, evaluations, intrapartum monitoring and transfer on respiratory support, surgery and follow-ups.Among them, 22 cases with severe disease and postnatal dyspnea received ex utero intrapartum treatment (EXIT).Clinical characteristics, surgical parameters and outcomes were compared.Results Among them, 62 survived and 33 died.Twenty-two received EXIT during labor, 21 were operated and 19 survived with a postoperative survival rate of 90.5%.There were 73 cases in non-EXIT group, 60 cases were operated and 43 cases survived with a postoperative survival rate of 71.7%.Logistic regression analysis showed that EXIT during labor (OR=0.104,[95%CI:0.022-0.493]), gestational age at prenatal diagnosis (OR=0.863,[95%CI:0.783-0.952]) and liver herniation into chest (OR=9.657,[95%CI:3.094-30.137]) were independent prognostic factors for CDH children.According to the receiver operating characteristic (ROC) curve, the cutoff value of maximizing the diagnostic efficiency of prenatal diagnostic age was 24.5 weeks.The survival rate of children with a gestational age under 24.5 weeks was significantly lower than that of those with a gestational age above 24.5 weeks.Conclusion Both safe and feasible, EXIT may improve the prognosis of neonatal CDH effectively.EXIT, gestational age of prenatal diagnosis and liver herniation into thoracic cavity are independent prognostic factors for CDH neonates.

References:

1 Politis MD,Bermejo-Sánchez E,Canfield MA,et al.Prevalence and mortality in children with congenital diaphragmatic hernia:a multicountry study[J].Ann Epidemiol,2021,56:61-69.e3.DOI:10.1016/j.annepidem.2020.11.007.
2 Chandrasekharan PK,Rawat M,Madappa R,et al.Congenital Diaphragmatic hernia-a review.Maternal health,neonatology and perinatology[J].2017,3:6.DOI:10.1186/s40748-017-0045-1.
3 Moldenhauer JS.Ex Utero Intrapartum Therapy.Seminars in pediatric surgery[J].2013,22(1):44-49.DOI:10.1053/j.sempedsurg.2012.10.008.
4 Lally KP,Lasky RE,Lally PA,et al.Standardized reporting for congenital diaphragmatic hernia-an international consensus[J].J Pediatr Surg,2013,48(12):2408-2415.DOI:10.1016/j.jpedsurg.2013.08.014.
5 Brindle ME,Cook EF,Tibboel D,et al.A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns[J].Pediatrics,2014,134(2):e413-419.DOI:10.1542/peds.2013-3367.
6 Coughlin MA,Werner NL,Gajarski R,et al.Prenatally diagnosed severe CDH:mortality and morbidity remain high[J].J Pediatr Surg,2016,51(7):1091-1095.DOI:10.1016/j.jpedsurg.2015.10.082.
7 Jani J,Keller RL,Benachi A,et al.Prenatal prediction of survival in isolated left-sided diaphragmatic hernia[J].Ultrasound Obstet Gynecol,2006,27(1):18-22.DOI:10.1002/uog.2688.
8 Akinkuotu AC,Cruz SM,Abbas PI,et al.Risk-stratification of severity for infants with CDH:Prenatal versus postnatal predictors of outcome[J].J Pediatr Surg,2016,51(1):44-48.DOI:10.1016/j.jpedsurg.2015.10.009.
9 Bouchghoul H,Senat MV,Storme L,et al.Congenital diaphragmatic hernia:does gestational age at diagnosis matter when evaluating morbidity and mortality?[J].Am J Obstet Gynecol,2015,213(4):535.e531-537.DOI:10.1016/j.ajog.2015.06.012.
10 Metkus AP,Filly RA,Stringer MD,et al.Sonographic predictors of survival in fetal diaphragmatic hernia[J].J Pediatr Surg,1996,31(1):148-151.DOI:10.1016/s0022-3468(96)90338-3.
11 Ruano R,Takashi E,da Silva MM,et al.Prediction and probability of neonatal outcome in isolated congenital diaphragmatic hernia using multiple ultrasound parameters[J].Ultrasound Obstet Gynecol,2012,39(1):42-49.DOI:10.1002/uog.10095.
12 Keijzer R,Liu J,Deimling J,et al.Dual-hit hypothesis explains pulmonary hypoplasia in the nitrofen model of congenital diaphragmatic hernia[J].Am J Pathol,2000,156(4):1299-1306.DOI:10.1016/s0002-9440(10)65000-6.
13 Skarsgard ED,Chitkara U,Krane EJ,et al.The OOPS procedure (operation on placental support):in utero airway management of the fetus with prenatally diagnosed tracheal obstruction[J].J Pediatr Surg,1996,31(6):826-828.DOI:10.1016/s0022-3468(96)90144-x.
14 Masahata K,Soh H,Tachibana K,et al.Clinical outcomes of ex utero intrapartum treatment for fetal airway obstruction[J].Pediatr Surg Int,2019,35(8):835-843.DOI:10.1007/s00383-019-04494-1.
15 Mychaliska GB,Bealer JF,Graf JL,et al.Operating on placental support:the ex utero intrapartum treatment procedure[J].J Pediatr Surg,1997,32(2):227-230.DOI:10.1016/s0022-3468(97)90184-6.
16 Flake AW,Crombleholme TM,Johnson MP,et al.Treatment of severe congenital diaphragmatic hernia by fetal tracheal occlusion:clinical experience with fifteen cases[J].Am J Obstet Gynecol,2000,183(5):1059-1066.DOI:10.1067/mob.2000.108871.
17 Harrison MR,Adzick NS,Flake AW,et al.Correction of congenital diaphragmatic hernia in utero VIII:Response of the hypoplastic lung to tracheal occlusion[J].J Pediatr Surg,1996,31(10):1339-1348.DOI:10.1016/s0022-3468(96)90824-6.
18 Bence CM,Wagner AJ.Ex utero intrapartum treatment (EXIT) procedures[J].Semin Pediatr Surg,2019,28(4):150820.DOI:10.1053/j.sempedsurg.2019.07.003.
19 Liechty KW,Crombleholme TM,Flake AW,et al.Intrapartum airway management for giant fetal neck masses:the EXIT (ex utero intrapartum treatment) procedure[J].Am J Obstet Gynecol,1997,177(4):870-874.DOI:10.1016/s0002-9378(97)70285-0.
20 Hirose S,Sydorak RM,Tsao K,et al.Spectrum of intrapartum management strategies for giant fetal cervical teratoma[J].J Pediatr Surg,2003,38(3):446-450.DOI:10.1053/jpsu.2003.50077.

Memo

收稿日期:2021-07-14。
基金项目:国家重点研发计划基金（编号：2018YFC1002503）；北京市卫生与健康科技成果与适宜技术推广项目（编号：BHTPP202005）；北京市儿科学科协同发展中心儿科重点专项基金（编号：XTZD20180305）
通讯作者:马立霜,malishuang2006@sina.com

Last Update: 1900-01-01