Surgical Management and Outcomes of Congenital Diaphragmatic Hernia in Neonates: A Systematic Review

Authors

  • Samina Ruquaya
  • Praveena Gaddam
  • Athira Gopinathan

Keywords:

Congenital diaphragmatic hernia, CDH, neonates, surgical intervention, thoracoscopic repair, ECMO, outcomes, fetal surgery, long-term morbidity, multidisciplinary care

Abstract

Congenital diaphragmatic hernia (CDH) is a severe neonatal disorder characterized by inadequate diaphragm development, leading to pulmonary hypoplasia and hypertension. Notwithstanding advancements in neonatal treatment, congenital diaphragmatic hernia (CDH) is linked to considerable morbidity and mortality. This systematic review assesses contemporary surgical techniques—spanning open and thoracoscopic repair to ECMO-assisted procedures—and their results in newborns with congenital diaphragmatic hernia (CDH). A comprehensive literature review was performed across various databases for studies published from 2000 to 2025. The research indicates that postponing surgical repair following preoperative stabilization results in superior outcomes compared to immediate surgery. Although minimally invasive surgery demonstrates potential for certain stable neonates, open repair continues to be the standard for critically ill patients. Patch repair is frequently essential for substantial flaws but entails increased chances of recurrence. The utilization of ECMO, while life-saving in critical instances, entails long-term neurodevelopmental hazards. Postoperative problems, including pulmonary hypertension, recurrence, and long-term morbidities such as chronic lung disease and developmental disabilities, persist. The prognosis is affected by elements including prenatal severity indicators, liver herniation, genetic abnormalities, and available institutional resources. The review highlights future directions, such as advancements in fetal surgery like FETO, the creation of novel surgical procedures, and the establishment of multidisciplinary follow-up programs to improve the quality of life for survivors. A customized, evidence-driven strategy is crucial for enhancing outcomes in infants with congenital diaphragmatic hernia (CDH).

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References

Albanese, C. T., Lopoo, J., Goldstein, R. B., Filly, R. A., Feldstein, V. A., Calen, P. W., Jennings, R. W., Farrell, J. A., & Harrison, M. R. 1998. Fetal liver location and perinatal outcomes in congenital diaphragmatic hernia. Prenatal Diagnosis, Volume 18, Issue 11, Pages 1138–1142. https://doi.org/10.1002/(sici)1097-0223(199811)18:11<1138: aid-pd416>3.0.co;2-a

Aly, H., Bianco-Batlles, D., Mohamed, M. A., & Hammad, T. A. 2010. Infant mortality associated with congenital diaphragmatic hernia: An analysis of the United States National Database. Journal of Perinatology, Volume 30, Issue 8, Pages 553–557. https://doi.org/10.1038/jp.2009.194

Costerus, S., Zahn, K., van de Ven, K., Vlot, J., Wessel, L., & Wijnen, R. 2016. Thoracoscopic versus open treatment of congenital diaphragmatic hernia in hemodynamically stable infants. Surgical Endoscopy, Volume 30, Issue 7, Pages 2818–2824. https://doi.org/10.1007/s00464-015-4560-8

Cox, K. J., Yang, M. J., Fenton, S. J., Russell, K. W., Yost, C. C., and Yoder, B. A. 2022. Timing of Surgical Intervention in Congenital Diaphragmatic Hernia: What is the Optimal Duration of Delay? Journal of Pediatric Surgery, Volume 57, Issue 9, Pages 17–23. https://doi.org/10.1016/j.jpedsurg.2022.01.020

Danzer, E., and Hedrick, H. L. 2011. Neurodevelopmental and neurofunctional results in children with congenital diaphragmatic hernia. Early Human Development, Volume 87, Issue 9, Pages 625–632. https://doi.org/10.1016/j.earlhumdev.2011.05.005

Deprest, J. A., Hyett, J. A., Flake, A. W., Nicolaides, K., and Gratacos, E. 2009. Current difficulties in prenatal diagnosis 4: Is fetal surgery warranted in all instances of severe diaphragmatic hernia? Prenatal Diagnosis, Volume 29, Issue 1, Pages 15–19. https://doi.org/10.1002/pd.2108

Deprest, J. A., Nicolaides, K. H., Benachi, A., Gratacos, E., Ryan, G., Persico, N., Sago, H., Johnson, A., Wielgoś, M., Berg, C., Van Calster, B., Russo, F. M., & TOTAL Trial for Severe Hypoplasia Investigators. 2021. Randomized trial of fetal surgery for severe left-sided diaphragmatic hernia. The New England Journal of Medicine, 385(2), 107–118. https://doi.org/10.1056/NEJMoa2027030

Desai, A. A., Ostlie, D. J., and Juang, D. 2015. Ideal timing for the surgical correction of congenital diaphragmatic hernia in newborns undergoing extracorporeal membrane oxygenation. Seminars in Pediatric Surgery, 24(1), 17–19. https://doi.org/10.1053/j.sempedsurg.2014.11.004

Gupta, V. S., Harting, M. T., Lally, P. A., Miller, C. C., Hirschl, R. B., Davis, C. F., Dassinger, M. S., Buchmiller, T. L., Van Meurs, K. P., Yoder, B. A., Stewart, M. J., Lally, K. P., and the Congenital Diaphragmatic Hernia Study Group. 2023. Mortality in congenital diaphragmatic hernia: A multicenter registry analysis of more than 5000 individuals over a span of 25 years. Annals of Surgery, Volume 277, Issue 3, Pages 520–527. https://doi.org/10.1097/SLA.0000000000005113.

Hollinger, L. E., Harting, M. T., & Lally, K. P. (2017). Prolonged monitoring of congenital diaphragmatic hernia. Seminars in Pediatric Surgery, Volume 26, Issue 3, Pages 178–184. https://doi.org/10.1053/j.sempedsurg.2017.04.007.

Kamata, S., Hasegawa, T., Ishikawa, S., Usui, N., Okuyama, H., Kawahara, H., Kubota, A., Fukuzawa, M., Imura, K., and Okada, A. 1992. Prenatal diagnosis of congenital diaphragmatic hernia and perinatal management: Evaluation of pulmonary hypoplasia. Early Human Development, 29(1–3), 375–379. https://doi.org/10.1016/0378-3782(92)90195-M

Keijzer, R., & Puri, P. (2010). Congenital diaphragmatic hernia. Seminars in Pediatric Surgery, Volume 19, Issue 3, Pages 180–185. https://doi.org/10.1053/j.sempedsurg.2010.03.001

Lum, L. C. S., Ramanujam, T. M., Yik, Y. I., Lee, M. L., Chuah, S. L., Breen, E., Zainal-Abidin, A. S., Singaravel, S., Thambidorai, C. R., de Bruyne, J. A., Nathan, A. M., Thavagnanam, S., Eg, K. P., Chan, L., Abdel-Latif, M. E., & Gan, C. S. 2022. Results of neonatal congenital diaphragmatic hernia in a non-ECMO facility inside a middle-income nation: A retrospective cohort analysis. BMC Pediatrics, Volume 22, Issue 1, Page 396. https://doi.org/10.1186/s12887-022-03453-5

Martino, A., Lista, G., & Guner, Y. S. 2022. Management of the congenital diaphragmatic hernia patient on extracorporeal life support. Seminars in Fetal and Neonatal Medicine, Volume 27, Issue 6, Article 101407. https://doi.org/10.1016/j.siny.2022.101407

Mattke, A. C., Alphonso, N., Ren, C., Jardine, L., Johnson, K. E., Venugopal, P., and McBride, C. A. 2025. Long-term results and quality of life in survivors of congenital diaphragmatic hernia treated with extracorporeal life support: A cross-sectional survey. Journal of Pediatrics and Child Health, 61(1), 75–79. https://doi.org/10.1111/jpc.16717.

Nguyen, T. L., and Le, A. D. 2006. Thoracoscopic correction of congenital diaphragmatic hernia: Insights from 45 patients. Journal of Pediatric Surgery, Volume 41, Issue 10, Pages 1713–1715. https://doi.org/10.1016/j.jpedsurg.2006.05.043

Numanoglu, A., Morrison, C., and Rode, H. 1998. Prognostication of results in congenital diaphragmatic hernia. Pediatric Surgery International, Volume 13, Issue 8, Pages 564–568. https://doi.org/10.1007/s003830050404.

Okawada, M., Ohfuji, S., Yamoto, M., Urushihara, N., Terui, K., Nagata, K., Taguchi, T., Hayakawa, M., Amari, S., Masumoto, K., Okazaki, T., Inamura, N., Toyoshima, K., Inoue, M., Furukawa, T., Yokoi, A., Kanamori, Y., Usui, N., Tazuke, Y., Saka, R., … Japanese Congenital Diaphragmatic Hernia Study Group. 2021. Thoracoscopic correction of congenital diaphragmatic hernia in neonates: Results from a multicenter trial in Japan. Surgery Today, Volume 51, Issue 10, Pages 1694–1702. https://doi.org/10.1007/s00595-021-02278-6

Okawada, M., Ohfuji, S., Yamoto, M., Urushihara, N., Terui, K., Nagata, K., Taguchi, T., Hayakawa, M., Amari, S., Masumoto, K., Okazaki, T., Inamura, N., Toyoshima, K., Inoue, M., Furukawa, T., Yokoi, A., Kanamori, Y., Usui, N., Tazuke, Y., Saka, R., … Japanese Congenital Diaphragmatic Hernia Study Group. 2021. Thoracoscopic correction of congenital diaphragmatic hernia in neonates: Results from a multicenter trial in Japan. Surgery Today, Volume 51, Issue 10, Pages 1694–1702. https://doi.org/10.1007/s00595-021-02278-6

Pollack, J. C., Hollinger, L. E., Buchmiller, T. L., and Jancelewicz, T. 2024. Prolonged monitoring in congenital diaphragmatic hernia. Seminars in Pediatric Surgery, Volume 33, Issue 4, Article 151443. https://doi.org/10.1016/j.sempedsurg.2024.151443

Snoek, K. G., Reiss, I. K. M., Greenough, A., Capolupo, I., Urlesberger, B., Wessel, L., Storme, L., Deprest, J., Schaible, T., van Heijst, A., & Tibboel, D. 2016. Standardized postnatal care of babies with congenital diaphragmatic hernia in Europe: The CDH EURO Consortium consensus – 2015 revision. Neonatology, Volume 110, Issue 1, Pages 66–74. https://doi.org/10.1159/000444210

Thodika, F. M. S. A., Dimitrova, S., Nanjundappa, M., Davenport, M., Nicolaides, K., Dassios, T., and Greenough, A. 2022. Forecasting survival in neonates with congenital diaphragmatic hernia and their reaction to breathed nitric oxide. European Journal of Pediatrics, Volume 181, Issue 10, Pages 3683–3689. https://doi.org/10.1007/s00431-022-04568-8

Veenma, D. C. M., de Klein, A., and Tibboel, D. 2012. Developmental and genetic factors of congenital diaphragmatic hernia. Pediatric Pulmonology, Volume 47, Issue 7, Pages 534–545. https://doi.org/10.1002/ppul.22553

Weller, K., Westra, D., Peters, N. C. J., Wilke, M., Van Opstal, D., Feenstra, I., van Drongelen, J., Eggink, A. J., Diderich, K. E. M., & DeKoninck, P. L. J. 2024. Exome sequencing in fetuses diagnosed with congenital diaphragmatic hernia within a national sample. Prenatal Diagnosis. https://doi.org/10.1002/pd.6622

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Published

2025-04-25

How to Cite

1.
Ruquaya S, Gaddam P, Gopinathan A. Surgical Management and Outcomes of Congenital Diaphragmatic Hernia in Neonates: A Systematic Review. J Neonatal Surg [Internet]. 2025Apr.25 [cited 2025Sep.19];14(6):632-40. Available from: https://www.jneonatalsurg.com/index.php/jns/article/view/4625