Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Review Article
  • Published:

Organization of care of infants with congenital diaphragmatic hernia—Building a high-functioning CDH program

Abstract

Infants born with congenital diaphragmatic hernia have high mortality and morbidity and require coordinated multidisciplinary care for optimal outcomes. Over the past several decades numerous articles have been published on the technical aspects of the care of these patients demonstrating both the variation in management across institutions as well as the desirability and need for standardization of care. Unfortunately, none have focused on the organization of care for CDH patients encompassing the range from early prenatal diagnosis to long-term postnatal care. However, to achieve optimal care and optimal outcomes, it is important to not only have excellent technical surgical and medical care but also to have an organized, systematic, and purposefully designed program for the delivery of healthcare to infants with this condition. In this article, based on our experience and drawing on general principles of building clinical programs, we describe the important elements of an ideal CDH program.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Fig. 1

Similar content being viewed by others

References

  1. Tovar JA. Congenital diaphragmatic hernia. Orphanet J Rare Dis. 2012;7. https://doi.org/10.1186/1750-1172-7-1.

  2. Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S. Congenital diaphragmatic hernia—a review. Matern Heal Neonatol Perinatol. 2017;3:532–40.

    Google Scholar 

  3. McHoney M. Congenital diaphragmatic hernia. Early Hum Dev. 2014;90:941–6.

    PubMed  Google Scholar 

  4. Doherty, Catherine H, MacKinnon RJ. Congenital diaphragmatic hernia-an update. Infant. 2006;2:244–8.

    Google Scholar 

  5. Hollinger LE, Harting MT, Lally KP. Long-term follow-up of congenital diaphragmatic hernia. Semin Pediatr Surg. 2017;26:178–84.

    PubMed  Google Scholar 

  6. Putnam LR, Harting MT, Tsao K, Morini F, Yoder BA, Luco M, et al. Congenital diaphragmatic hernia defect size and infant morbidity at discharge. Pediatrics. 2016;138:11.

    Google Scholar 

  7. Peetsold MG, Heij HA, Kneepkens CM, Nagelkerke AF, Huisman J, Gemke RJ. The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity. Pediatr Surg Int. 2009;25:1–17.

    CAS  PubMed  Google Scholar 

  8. Morini F, Valfrè L, Bagolan P. Long-term morbidity of congenital diaphragmatic hernia: a plea for standardization. Semin Pediatr Surg. 2017;26:301–10.

    PubMed  Google Scholar 

  9. Lally PA, Skarsgard ED. Congenital diaphragmatic hernia: the role of multi-institutional collaboration and patient registries in supporting best practice. Semin Pediatr Surg. 2017;26:129–35.

    PubMed  Google Scholar 

  10. Snoek KG, Reiss IKM, Greenough A, Capolupo I, Urlesberger B, Wessel L, et al. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium Consensus—2015 update. Neonatology. 2016;110:66–74.

    PubMed  Google Scholar 

  11. Antonoff MB, Hustead VA, Groth SS, Schmeling DJ. Protocolized management of infants with congenital diaphragmatic hernia: effect on survival. J Pediatr Surg. 2011;46:39–46.

    PubMed  Google Scholar 

  12. Reiss I, Schaible T, Van Den Hout L, Capolupo I, Allegaert K, Van Heijst A, et al. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium consensus. Neonatology. 2010;98:354–64.

    CAS  PubMed  Google Scholar 

  13. Peralta CF, Lourenço PR, Lopes PN, Baptista C, Pais L. Team development: definition, measurement and relationships with team effectiveness. Hum Perform. 2018;31:97–124. https://doi.org/10.1080/08959285.2018.1455685.

  14. Markiewicz L, West MA. Effective Team Working in Health Care. In The Oxford Handbook of Health Care Management. Oxford University Press; 2016. https://doi.org/10.1093/oxfordhb/9780198705109.013.8.

  15. Kozlowski SWJ, Ilgen DR. Enhancing the effectiveness of work groups and teams. Psychol Sci Public Interest Suppl. 2006;7:77–124.

    Google Scholar 

  16. Tannenbaum S, Salas E. “Teams That Work.” In Teams That Work. United States: Oxford University Press, Incorporated, 2020.

  17. Weller J, Boyd M, Cumin D. Teams, tribes and patient safety: overcoming barriers to effective teamwork in healthcare. Postgrad Med J. 2014;90:149–54.

    PubMed  Google Scholar 

  18. Stout S, Zallman L, Arsenault L, Sayah A, Hacker K. Developing high-functioning teams: factors associated with operating as a ‘real team’ and implications for patient-centered medical home development. Inquiry. 2017;54:1–9.

    Google Scholar 

  19. Abraham TH, Stewart GL, Solimeo SL. The importance of soft skills development in a hard data world: learning from interviews with healthcare leaders. BMC Med Educ. 2021;21. https://doi.org/10.1186/S12909-021-02567-1.

  20. Willard-Grace R, Hessler D, Rogers E, Dubé K, Bodenheimer T, Grumbach K. Team structure and culture are associated with lower burnout in primary care. J Am Board Fam Med. 2014;27:229–38.

    PubMed  Google Scholar 

  21. Azad N, Anderson HG, Brooks A, Garza O, O’Neil C, Stutz MM, et al. Leadership and management are one and the same. Am J Pharm Educ. 2017;81. https://doi.org/10.5688/AJPE816102.

  22. Hicks CW, Rosen M, Hobson DB, Ko C, Wick EC. Improving safety and quality of care with enhanced teamwork through operating room briefings. JAMA Surg. 2014;149:863–8.

    PubMed  Google Scholar 

  23. Rassi JA, Fino-Szumski MS. Vision, values, and mission: foundations for quality improvement. Semin Hear. 1994;15:267–76.

    Google Scholar 

  24. Kirkpatrick SA. Understanding the role of vision, mission, and values in the HPT model. Perform Improv. 2017;56:6–14.

    Google Scholar 

  25. Guerguerian A-M, Ogino MT, Dalton HJ, Shekerdemian LS. Setup and maintenance of extracorporeal life support programs. Pediatr Crit Care Med. 2013;14:S84–93.

    PubMed  Google Scholar 

  26. Moher D, Weinberg A, Hanlon R, Runnalls K. Effects of a medical team coordinator on length of hospital stay. Can Med Assoc J. 1992;146:511.

    CAS  Google Scholar 

  27. Salas E, Weaver SJ, Lyons R, Diazgranados D, Rosen MA, Oglesby J, et al. The anatomy of health care team training and the state of practice: a critical review. Acad Med. 2010;85:1746–60.

    PubMed  Google Scholar 

  28. Cates LA. Simulation training: a multidisciplinary approach. Adv Neonatal Care. 2011;11:95–100.

    PubMed  Google Scholar 

  29. Cheng A, Donoghue A, Gilfoyle E, Eppich W. Simulation-based crisis resource management training for pediatric critical care medicine: a review for instructors. Pediatr Crit Care Med. 2012;13:197–203.

    PubMed  Google Scholar 

  30. Salas Professor E, Cannon-Bowers J, Johnston JH. Chapter 33. In: Zsambok CE, Klein G. editors. Naturalistic Decision Making. 1st ed. New York: Psychology Press; 1997. https://doi.org/10.4324/9781315806129.

  31. Salas E, DiazGranados D, Weaver SJ, King H. Does team training work? Principles for health care. Acad Emerg Med. 2008;15:1002–9.

    PubMed  Google Scholar 

  32. Greilich PE, Kilcullen M, Paquette S, Lazzara EH, Scielzo S, Hernandez J, et al. Team FIRST framework: Identifying core teamwork competencies critical to interprofessional healthcare curricula. J Clin Transl Sci. 2023;7:106–7.

    Google Scholar 

  33. ELSO Course and Workshops. 2023. https://www.elso.org/ecmo-education/endorsed-ecmo-courses.aspx.

  34. Patel J, Posencheg M, Ades A. Proficiency and retention of neonatal resuscitation skills by pediatric residents. Pediatrics. 2012;130:515–21.

    PubMed  Google Scholar 

  35. History of the Neonatal Resuscitation Program. 2023. https://www.aap.org/en/learning/neonatal-resuscitation-program/nrp-history/.

  36. Horbar JD. The vermont oxford network: evidence-based quality improvement for neonatology. Pediatrics. 1999;103:350–9.

    CAS  PubMed  Google Scholar 

  37. Horbar JD, Rogowski J, Plsek PE, Delmore P, Edwards WH, Hocker J, et al. Collaborative quality improvement for neonatal intensive care. NIC/Q Project Investigators of the Vermont Oxford Network. Pediatrics. 2001;107:14–22.

    CAS  PubMed  Google Scholar 

  38. ELSO Award of Excellence in ECMO | Extracorporeal Membrane Oxygenation. 2023. https://www.elso.org/awardofexcellence.aspx.

  39. Lum L, Kervin J, Clark K, Reid F, Sirola W. Explaining nursing turnover intent: job satisfaction, pay satisfaction, or organizational commitment? *. J Organ Behav. 1998;19:305–20.

    Google Scholar 

  40. Dietrichson J, Gudmundsson J, Jochem T. Why don’t we talk about it? Communication and coordination in teams. J Econ Behav Organ. 2022;197:257–78. https://doi.org/10.1016/j.jebo.2022.02.018.

  41. Reader TW, Flin R, Cuthbertson BH. Communication skills and error in the intensive care unit. Curr Opin Crit Care. 2007;13:732–6.

    PubMed  Google Scholar 

  42. The Canadian CDH Collaborative on the App Store. 2023. https://apps.apple.com/us/app/the-canadian-cdh-collaborative/id1437160471.

  43. Guner Y, Jancelewicz T, Di Nardo M, Yu P, Brindle M, Vogel AM, et al. Management of congenital diaphragmatic hernia treated with extracorporeal life support: interim guidelines consensus statement from the Extracorporeal Life Support Organization. ASAIO J. 2021;67:113–20.

    CAS  PubMed  Google Scholar 

  44. Rosen P, Stenger E, Bochkoris M, Hannon MJ, Kwoh K. Family-centered multidisciplinary rounds enhance the team approach in pediatrics. Pediatrics. 2009;123:e603–8.

    PubMed  Google Scholar 

  45. Clay-Williams R, Plumb J, Luscombe GM, Hawke C, Dalton H, Shannon G, et al. Improving teamwork and patient outcomes with daily structured interdisciplinary bedside rounds: a multimethod evaluation. J Hosp Med. 2018;13:311.

    PubMed  Google Scholar 

  46. Henkin S, Chon TY, Christopherson ML, Halvorsen AJ, Worden LM, Ratelle JT. Improving nurse-physician teamwork through interprofessional bedside rounding. J Multidiscip Health. 2016;9:201.

    Google Scholar 

  47. Goldstein RF, Malcolm WF. Care of the neonatal intensive care unit graduate after discharge. Pediatr Clin North Am. 2019;66:489–508.

    PubMed  Google Scholar 

  48. Tragesser CJ, Hafezi N, Kitsis M, Markel TA, Gray BW. Survivors of congenital diaphragmatic hernia repair face barriers to long-term follow-up care. J Surg Res. 2021;267:243–50.

    PubMed  Google Scholar 

  49. Clinical Practice Guidelines | Pediatrics | American Academy of Pediatrics; 2022. https://publications.aap.org/pediatrics/collection/523/Clinical-Practice-Guidelines.

  50. Guyatt G, Oxman AD, Akl EA, Kunz R, Vist G, Brozek J, et al. GRADE guidelines: 1. Introduction—GRADE evidence profiles and summary of findings tables. J Clin Epidemiol. 2011;64:383–94.

    PubMed  Google Scholar 

  51. Petkovic J, Riddle A, Akl EA, Khabsa J, Lytvyn L, Atwere P, et al. Protocol for the development of guidance for stakeholder engagement in health and healthcare guideline development and implementation. Syst Rev. 2020;9:1–11.

    Google Scholar 

  52. Traversy G, Barnieh L, Akl EA, Allan GM, Brouwers M, Ganache I, et al. Managing conflicts of interest in the development of health guidelines. CMAJ. 2021;193:E49–54.

    PubMed  PubMed Central  Google Scholar 

  53. Woolf SH, Grol R, Hutchinson A, Eccles M, Grimshaw J. Clinical guidelines. Potential benefits, limitations, and harms of clinical guidelines. Br Med J. 1999;318:527–30.

    CAS  Google Scholar 

  54. Bain K, Hansen AS. Strengthening implementation success using large-scale consensus decision-making—a new approach to creating medical practice guidelines. Eval Program Plan. 2020;79:101730.

    Google Scholar 

  55. Tetreault L, Nater A, Garwood P, Badhiwala JH, Wilson JR, Fehlings MG. Development and implementation of clinical practice guidelines: an update and synthesis of the literature with a focus in application to spinal conditions. Glob Spine J. 2019;9:53S–64S.

    Google Scholar 

  56. Gupta M, Kaplan HC. Measurement for quality improvement: using data to drive change. J Perinatol. 2020;40:962–71.

    PubMed  Google Scholar 

  57. Ratner S, Pignone M. Quality improvement principles and practice. Prim Care. 2019;46:505–14.

    PubMed  Google Scholar 

  58. Grol R, Grimshaw J. From best evidence to best practice: effective implementation of change in patients’ care. Lancet. 2003;362:1225–30.

    PubMed  Google Scholar 

  59. Ovretveit J, Dolan-Branton L, Marx M, Reid A, Reid J, Agins B. Adapting improvements to context: when, why and how? Int J Qual Health Care. 2018;30:20–23.

    PubMed  PubMed Central  Google Scholar 

  60. Bradley EH, Holmboe ES, Mattera JA, Roumanis SA, Radford MJ, Krumholz HM, et al. Data feedback efforts in quality improvement: lessons learned from US hospitals. Qual Saf Health Care. 2004;13:26–31.

    CAS  PubMed  PubMed Central  Google Scholar 

  61. Samuel JP, Burgart A, Wootton SH, Magnus D, Lantos JD, Tyson JE. Randomized n-of-1 trials: quality improvement, research, or both? 2016. https://doi.org/10.1542/peds.2016-1103.

  62. Selker HP, Cohen T, D’Agostino RB, Dere WH, Ghaemi SN, Honig PK, et al. A useful and sustainable role for N-of-1 trials in the healthcare ecosystem. Clin Pharm Ther. 2022;112:224–32.

    Google Scholar 

  63. Shaughnessy AF, Cosgrove L, Lexchin JR. The need to systematically evaluate clinical practice guidelines. J Am Board Fam Med. 2016;29:644–8. https://doi.org/10.3122/jabfm.2016.06.160115.

  64. Sutton RT, Pincock D, Baumgart DC, Sadowski DC, Fedorak RN, Kroeker KI. An overview of clinical decision support systems: benefits, risks, and strategies for success. npj Digital Med. 2020;3:1–10.

    Google Scholar 

  65. Hagadorn JI, Johnson KR, Hill D, Sink DW. Improving the quality of quality metrics in neonatology. Semin Perinatol. 2020;44. https://doi.org/10.1016/J.SEMPERI.2020.151244.

  66. Ellsbury DL, Clark RH, Ursprung R, Handler DL, Dodd ED, Spitzer AR. A multifaceted approach to improving outcomes in the NICU: the pediatrix 100 000 babies campaign. Pediatrics. 2016;137. https://doi.org/10.1542/peds.2015-0389.

  67. Ganapathy V, Hay JW, Kim JH. Costs of necrotizing enterocolitis and cost-effectiveness of exclusively human milk-based products in feeding extremely premature infants. Breastfeed Med. 2012;7:29–37.

    PubMed  Google Scholar 

  68. Murthy K, Dykes FD, Padula MA, Pallotto EK, Reber KM, Durand DJ, et al. The children’s hospitals neonatal database: an overview of patient complexity, outcomes and variation in care. J Perinatol. 2014;34:582–6.

    CAS  PubMed  Google Scholar 

  69. Enticott J, Braaf S, Johnson A, Jones A, Teede HJ. Leaders’ perspectives on learning health systems: a qualitative study. BMC Health Serv Res. 2020;20:1–13.

    Google Scholar 

  70. Reyes DL, Bisbey T, Day D, Salas E. Translating 6 key insights from research on leadership and management in times of crisis. BMJ Lead. 2021;0:1–4.

    Google Scholar 

  71. Prang KH, Maritz R, Sabanovic H, Dunt D, Kelaher M. Mechanisms and impact of public reporting on physicians and hospitals’ performance: a systematic review (2000-2020). PLoS One. 2021;16. https://doi.org/10.1371/journal.pone.0247297.

  72. Akinkuotu AC, Cruz SM, Abbas PI, Lee TC, Welty SE, Olutoye OO, et al. Risk-stratification of severity for infants with CDH: prenatal versus postnatal predictors of outcome. J Pediatr Surg. 2016;51:44–8.

  73. Lewit RA, Jancelewicz T. Sources of regional and center-level variability in survival and cost of care for congenital diaphragmatic hernia (CDH). J Pediatr Surg. 2021;56:130–5.

    PubMed  Google Scholar 

  74. Jancelewicz T, Brindle ME, Harting MT, Tolley EA, Langham MR, Lally PA, et al. Extracorporeal membrane oxygenation (ECMO) risk stratification in newborns with congenital diaphragmatic hernia (CDH). J Pediatr Surg. 2018;53:1890–5.

    PubMed  Google Scholar 

  75. Guner YS, Harting MT, Jancelewicz T, Yu PT, Di Nardo M, Nguyen DV. Variation across centers in standardized mortality ratios for congenital diaphragmatic hernia receiving extracorporeal life support. J Pediatr Surg. 2022;57:606–13. https://doi.org/10.1016/j.jpedsurg.2022.01.022.

    Article  PubMed  Google Scholar 

  76. Soltys FC, Spilo K, Politi MC. The content and quality of publicly available information about congenital diaphragmatic hernia: descriptive study. JMIR Pediatr Parent. 2021;4:e30695. https://doi.org/10.2196/30695.

  77. Russo FM, Debeer A, De Coppi P, Devriendt K, Crombag N, Hubble T, et al. What should we tell parents? Congenital diaphragmatic hernia. 2020. https://doi.org/10.1002/pd.5880.

  78. Crombag N, Ceulemans V, Debeer A, Russo F, Bollen B, Power B, et al. Prenatal diagnosis of congenital diaphragmatic hernia: parental counselling and support needs. Prenat Diagn. 2022;42:387–97.

    CAS  PubMed  Google Scholar 

  79. Tsao K, Johnson A. Fetal tracheal occlusion for congenital diaphragmatic hernia. Semin Perinatol. 2020;44:151164.

    PubMed  Google Scholar 

  80. Outcomes of CDH at Texas Children’s Hospital. 2023. https://women.texaschildrens.org/program/texas-childrens-fetal-center/volumes-outcomes/congenital-diaphragmatic-hernia-cdh.

  81. Outcomes of CDH Survivors at Children’s Hospital of Philadelphia. 2023. https://www.chop.edu/news/outcomes-cdh-survivors.

  82. Horn-Oudshoorn EJJ, Peters NCJ, Franx A, Eggink AJ, Cochius-den Otter SCM, Reiss IKM, et al. Termination of pregnancy after a prenatal diagnosis of congenital diaphragmatic hernia: factors influencing the parental decision process. Prenat Diagn. 2023;43:95–101.

    CAS  PubMed  Google Scholar 

  83. Carter BS, Hubble C, Weise KL. Palliative medicine in neonatal and pediatric intensive care. Child Adolesc Psychiatr Clin N Am. 2006;15:759–77.

    PubMed  Google Scholar 

  84. Weise KL, Okun AL, Carter BS, Christian CW. Guidance on forgoing life-sustaining medical treatment. Pediatrics (Evanston). 2017;140. https://doi.org/10.1542/peds.2017-1905.

  85. Harting MT, Munson D, Linebarger J, Hirshberg E, Gow KW, Malek MM, et al. Ethical considerations in critically Ill neonatal and pediatric patients*. 2023. https://doi.org/10.1016/j.jpedsurg.2023.02.009.

  86. Donabedian A. Evaluating the quality of medical care. Milbank Q. 2005;83:691–729.

    PubMed  PubMed Central  Google Scholar 

  87. Fischer S. Hospital positioning and integrated hospital marketing communications: state-of-the-art review, conceptual framework, and research agenda. J Nonprofit Public Sect Mark. 2014;26:1–34. https://doi.org/10.1080/10495142.2014.870431.

  88. Huerta TR, Walker DM, Ford EW. An evaluation and ranking of children’s hospital websites in the United States. J Med Internet Res. 2016;18:e228. https://www.jmir.org/2016/8/e228.

    PubMed  PubMed Central  Google Scholar 

  89. Patient Stories. 2023. https://waystogive.texaschildrens.org/reasons-to-give/patient-stories/.

  90. Weller S. 6 inspirational medical stories of patient perseverance and resilience. Johnson & Johnson; 2023. https://www.jnj.com/personal-stories/6-inspirational-medical-stories-of-patient-perseverance-and-resilience.

  91. Anon. Groundbreaking Procedure for Severe CDH | Texas Children’s Pavilion for Women; 2023. https://women.texaschildrens.org/program/texas-childrens-fetal-center/patient-stories/torry-family.

  92. Marshall MN, Shekelle PG, Davies HTO, Smith PC. Public reporting on quality in the United States and the United Kingdom. Health Aff. 2017;22:134–48. https://doi.org/10.1377/hlthaff.22.3.134.

  93. Hedrick HL, Adzick NS. Congenital diaphragmatic hernia in the neonate—UpToDate. 2023. https://www.uptodate.com/contents/congenital-diaphragmatic-hernia-in-the-neonate.

  94. Tracy S, Chen C. Multidisciplinary long-term follow-up of congenital diaphragmatic hernia: a growing trend. 2014. https://doi.org/10.1016/j.siny.2014.09.001.

  95. Hollinger LE, Buchmiller TL. Long term follow-up in congenital diaphragmatic hernia. Semin Perinatol. 2020;44:151171.

    PubMed  Google Scholar 

  96. Section on Surgery and the Committee on Fetus and Newborn. Postdischarge follow-up of infants with congenital diaphragmatic hernia. Pediatrics. 2008;121:627–32.

    Google Scholar 

  97. Pierro M, Thébaud B. Understanding and treating pulmonary hypertension in congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 2014;19:357–63.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Contributions

CJF conceived and designed the work that led to the submission, acquired the data, drafted the initial manuscript, revised subsequent iterations, and approved the final version. KSG conceptualized the outline and designed the paper, critically reviewed and revised the manuscript for important intellectual content, and approved the final manuscript. Both authors agree to be accountable for all aspects of the work.

Corresponding author

Correspondence to Caraciolo J. Fernandes.

Ethics declarations

Competing interests

The authors declare no competing interests.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Fernandes, C.J., Gautham, K.S. Organization of care of infants with congenital diaphragmatic hernia—Building a high-functioning CDH program. J Perinatol 44, 339–347 (2024). https://doi.org/10.1038/s41372-023-01789-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/s41372-023-01789-z

Search

Quick links