Advertisement

Pediatric Surgery International

, Volume 35, Issue 3, pp 303–313 | Cite as

Congenital diaphragmatic hernia: the good, the bad, and the tough

  • Emrah AydinEmail author
  • Foong-Yen Lim
  • Paul Kingma
  • Beth Haberman
  • Beth Rymeski
  • Patricia Burns
  • Jose L. Peiro
Original Article

Abstract

Objectives

We aim to determine factors that are associated with better outcomes of CDH patients.

Methods

A retrospective review was performed on all CDH patients admitted to our institution between 2003 and 2016. This study was performed at a single institution which has a fetal care center. Patients admitted with CDH with at least 1-year follow-up during the analysis were included in the study.

Results

Twenty-six (13.8%) patients had a hernia sac, 124 (59%) patients had liver herniation, and 56 (25.1%) patients had an accompanying syndrome. Overall survival to discharge was 73.1% while overall survival to date was 69.5%. The presence of a hernia sac, liver herniation, and accompanying syndromes showed as independent predictors influencing the survival, B 1.968, p = 0.04, OR 7.158, 95% CI 0.907–56.485, B − 1.178, p = 0.01, OR 3.932, 95% CI 1.798–8.602 and B − 1.032, p = 0.05, OR 2.795, 95% CI 0.976–7.764, respectively.

Conclusion

In our CDH cohort, the presence of a hernia sac was proven to be associated with better outcomes, while thoracic herniation of the liver was associated with worse outcomes. The accompanying syndromes although being more difficult to manage had a little effect on the outcome of the disease itself.

Keywords

Congenital diaphragmatic hernia Hernia sac Liver herniation Syndrome Prognostic factors Cardiac anomalies 

Notes

Author contributions

All authors have made substantial contributions to the conception and design of the study, acquisition, analysis, and interpretation of data, drafting the article and revising it for important intellectual content and final approval of the version to be submitted.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Compliance with ethical standards

Conflict of interest

The authors report no proprietary or commercial interest in any product mentioned or concept discussed in this article.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (IRB #2017-6361) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

References

  1. 1.
    Zani A, Zani-Ruttenstock E, Pierro A (2014) Advances in the surgical approach to congenital diaphragmatic hernia. Semin Fetal Neonatal Med 19:364–369.  https://doi.org/10.1016/j.siny.2014.09.002 CrossRefGoogle Scholar
  2. 2.
    Tovar JA, Irish M, Holm B et al (2012) Congenital diaphragmatic hernia. Orphanet J Rare Dis 7:1.  https://doi.org/10.1186/1750-1172-7-1 CrossRefGoogle Scholar
  3. 3.
    Logan J, Rice H, Goldberg R, Cotten C (2007) Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies. J Perinatol 27:535–549CrossRefGoogle Scholar
  4. 4.
    Keijzer R, Puri P (2010) Congenital diaphragmatic hernia. Semin Pediatr Surg 19:180–185.  https://doi.org/10.1053/j.sempedsurg.2010.03.001 CrossRefGoogle Scholar
  5. 5.
    Grizelj R, Bojanić K, Vuković J et al (2017) Hernia sac presence portends better survivability of isolated congenital diaphragmatic hernia with “Liver-Up”. Am J Perinatol 34:515–519.  https://doi.org/10.1055/s-0036-1593765 CrossRefGoogle Scholar
  6. 6.
    Zamora IJ, Cass DL, Lee TC et al (2013) The presence of a hernia sac in congenital diaphragmatic hernia is associated with better fetal lung growth and outcomes. J Pediatr Surg 48:1165–1171.  https://doi.org/10.1016/j.jpedsurg.2013.03.010 CrossRefGoogle Scholar
  7. 7.
    Spaggiari E, Stirnemann J, Bernard JP et al (2013) Prognostic value of a hernia sac in congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 41:286–290.  https://doi.org/10.1002/uog.11189 CrossRefGoogle Scholar
  8. 8.
    Zerhouni S, Mayer C, Skarsgard ED (2013) Can we select fetuses with intra-abdominal calcification for delivery in neonatal surgical centres? J Pediatr Surg 48:946–950.  https://doi.org/10.1016/j.jpedsurg.2013.02.006 CrossRefGoogle Scholar
  9. 9.
    Albanese CT, Lopoo J, Goldstein RB et al (1998) Fetal liver position and perinatal outcome for congenital diaphragmatic hernia. Prenat Diagn 18:1138–1142CrossRefGoogle Scholar
  10. 10.
    Fumino S, Shimotake T, Kume Y et al (2005) A clinical analysis of prognostic parameters of survival in children with congenital diaphragmatic hernia. Eur J Pediatr Surg 15:399–403.  https://doi.org/10.1055/s-2005-872925 CrossRefGoogle Scholar
  11. 11.
    Jani J, Keller RL, Benachi A et al (2006) Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Ultrasound Obstet Gynecol 27:18–22.  https://doi.org/10.1002/uog.2688 CrossRefGoogle Scholar
  12. 12.
    Kitano Y, Nakagawa S, Kuroda T et al (2005) Liver position in fetal congenital diaphragmatic hernia retains a prognostic value in the era of lung-protective strategy. J Pediatr Surg 40:1827–1832CrossRefGoogle Scholar
  13. 13.
    Hedrick HL, Danzer E, Merchant A et al (2007) Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia. Am J Obstet Gynecol.  https://doi.org/10.1016/j.ajog.2007.07.001 Google Scholar
  14. 14.
    Cannie M, Jani J, Chaffiotte C et al (2008) Quantification of intrathoracic liver herniation by magnetic resonance imaging and prediction of postnatal survival in fetuses with congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 32:627–632.  https://doi.org/10.1002/uog.6146 CrossRefGoogle Scholar
  15. 15.
    Ruano R, Lazar DA, Cass DL et al (2014) Fetal lung volume and quantification of liver herniation by magnetic resonance imaging in isolated congenital diaphragmatic hernia. Ultrasound Obstet Gynecol.  https://doi.org/10.1002/uog.13223 Google Scholar
  16. 16.
    Jani JC, Benachi A, Nicolaides KH et al (2009) Prenatal prediction of neonatal morbidity in survivors with congenital diaphragmatic hernia: a multicenter study. Ultrasound Obstet Gynecol 33:64–69.  https://doi.org/10.1002/uog.6141 CrossRefGoogle Scholar
  17. 17.
    Grethel EJ, Farrell J, Ball R et al (2007) Congenital diaphragmatic hernia associated with spinal anomalies. Obstet Gynecol 109:485–488CrossRefGoogle Scholar
  18. 18.
    Sydorak RM, Goldstein R, Hirose S et al (2002) Congenital diaphragmatic hernia and hydrops: a lethal association? J Pediatr Surg 37(12):1678–1680CrossRefGoogle Scholar
  19. 19.
    Fauza DO, Wilson JM (1994) Congenital diaphragmatic hernia and associated anomalies: their incidence, identification, and impact on prognosis. J Pediatr Surg 29:1113–1117.  https://doi.org/10.1016/0022-3468(94)90290-9 CrossRefGoogle Scholar
  20. 20.
    Danzer E, Hedrick HL (2014) Controversies in the management of severe congenital diaphragmatic hernia. Semin Fetal Neonatal Med 19:376–384.  https://doi.org/10.1016/j.siny.2014.10.001 CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Pediatric General and Thoracic Surgery, The Center for Fetal Cellular and Molecular TherapyCincinnati Fetal CenterCincinnatiUSA
  2. 2.Division of Neonatology and Pulmonary BiologyCincinnati Children’s Hospital Medical CenterCincinnatiUSA

Personalised recommendations