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.

  • Original Article
  • Published:

Predicting death or extended length of stay in infants with congenital diaphragmatic hernia

Abstract

Objective:

To predict mortality or length of stay (LOS) >109 days (90th percentile) among infants with congenital diaphragmatic hernia (CDH).

Study Design:

We conducted a retrospective analysis using the Children's Hospital Neonatal Database during 2010 to 2014. Infants born >34 weeks gestation with CDH admitted at 22 participating regional neonatal intensive care units were included; patients who were repaired or were at home before admission were excluded. The primary outcome was death before discharge or LOS >109 days. Factors associated with this outcome were used to develop a multivariable equation using 80% of the cohort. Validation was performed in the remaining 20% of infants.

Results:

The median gestation and age at referral in this cohort (n=677) were 38 weeks and 6 h, respectively. The primary outcome occurred in 242 (35.7%) infants, and was distributed between mortality (n=180, 27%) and LOS >109 days (n=66, 10%). Regression analyses showed that small for gestational age (odds ratio (OR) 2.5, P=0.008), presence of major birth anomalies (OR 5.9, P<0.0001), 5- min Apgar score 3 (OR 7.0, P=0.0002), gradient of acidosis at the time of referral (P<0.001), the receipt of extracorporeal support (OR 8.4, P<0.0001) and bloodstream infections (OR 2.2, P=0.004) were independently associated with death or LOS >109 days. This model performed well in the validation cohort (area under curve (AUC)=0.856, goodness-of-fit (GF) χ2, P=0.16) and acted similarly even after omitting extracorporeal support (AUC=0.82, GF χ2, P=0.05).

Conclusions:

Six variables predicted death or LOS 109 days in this large, contemporary cohort with CDH. These results can assist in risk adjustment for comparative benchmarking and for counseling affected families.

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

Figure 1

Similar content being viewed by others

References

  1. Badillo A, Gingalewski C . Congenital diaphragmatic hernia: treatment and outcomes. Semin Perinatol 2014; 38 (2): 92–96.

    Article  Google Scholar 

  2. Haroon J, Chamberlain RS . An evidence-based review of the current treatment of congenital diaphragmatic hernia. Clin Pediatr (Phila) 2013; 52 (2): 115–124.

    Article  Google Scholar 

  3. Bohn D . Congenital diaphragmatic hernia. Am J Respir Crit Care Med 2002; 38: 729–732.

    Google Scholar 

  4. Wynn J, Krishnan U, Aspelund G, Zhang Y, Duong K, Stolar CJH et al. Outcomes of congenital diaphragmatic hernia in the modern era of management. J Pediatr 2013; 163: 114–119e1.

    Article  Google Scholar 

  5. Madenci AL, Sjogren AR, Treadwell MC, Ladin-Torres MF, Drongowski RA, Kreutzman J et al. Another dimension to survival: predicting outcomes with fetal MRI versus prenatal ultrasound in patients with congenital diaphragmatic hernia. J Pediatr Surg 2013; 48 (6): 1190–1197.

    Article  Google Scholar 

  6. Alfaraj MA, Shah PS, Bohn D, Pantazi S, O’Brien K, Chiu PP et al. Congenital diaphragmatic hernia: lung-to-head ratio and lung volume for prediction of outcome. Am J Obstet Gynecol 2011; 205 (43): e1–e8.

    Google Scholar 

  7. Steurer MA, Moon-Grady AJ, Fineman JR, Sun EC, Lusk LA, Wai KC et al. B-type natriuretic peptide: prognostic marker in congenital diaphragmatic hernia. Pediatr Res 2014; 76 (6): 549–554.

    Article  CAS  Google Scholar 

  8. Brindle ME, Cook EF, Tibboel D, Lally PA, Lally KP, CDH Study Group. A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns. Pediatr 2014; 134 (2): e413–e419.

    Article  Google Scholar 

  9. Schultz CM, DiGeronimo RJ, Yoder BA, CDH Study Group. Congenital diaphragmatic hernia: a simplified postnatal predictor of outcome. J Pediatr Surg 2007; 42: 510–516.

    Article  Google Scholar 

  10. Baird R, Eeson G, Safavi A, Puligandla P, Laberge JM, Skarsgard E . Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: a report from the Canadian Pediatric Surgery Network. J Pediatr Surg 2011; 46: 801–807.

    Article  Google Scholar 

  11. 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 (8): 582–586.

    Article  CAS  Google Scholar 

  12. Grover TR, Murthy K, Brozanski B, Gien J, Rintoul N, Keene S et al. Short-term outcomes and medical and surgical interventions in infants with congenital diaphragmatic hernia. Am J Perinatol 2015; 32 (11): 1038–1044.

    Article  Google Scholar 

  13. Rais-Bahrami K, Van Meurs KP . Venoarterial versus venovenous ECMO for neonatal respiratory failure. Semin Perinatol 2014; 38 (2): 71–77.

    Article  Google Scholar 

  14. Olsen IE, Groveman SA, Lawson ML, Clark RH, Zemel BS . New intrauterine growth curves based on United States data. Pediatrics 2010; 125 (2): e214–e224.

    Article  Google Scholar 

  15. Horan TC, Andrus M, Dudeck MA . CDC/NHSN surveillance definition of health care-associated infection and criteria for specific types of infections in the acute care setting. Am J Infect Control 2008; 35: 309–332.

    Article  Google Scholar 

  16. Seetharamaiah R, Younger JG, Bartlett RH, Hirschl RB, CDH Study Group. Factors associated with survival in infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 2009; 44: 1315–1321.

    Article  Google Scholar 

  17. Mah VK, Chiu P, Kim PC . Are we making a real difference? Update on ‘hidden mortality’ in the management of congenital diaphragmatic hernia. Fetal Diagn Ther 2011; 29 (1): 40–45.

    Article  Google Scholar 

  18. International Summary Extracorporeal Life Support Organization. Registry Report of the Extracorporeal Life Support Organization. International Summary Extracorporeal Life Support Organization: Ann Arbor, MI, USA, 2008.

  19. Stevens TP, van Wijngaarder E, Ackerman KG, Lally PA, Lally KP, CDH Study Group. Timing of delivery and survival rates for babies with prenatal diagnoses of congenital diaphragmatic hernia. Pediatr 2009; 123 (2): 494–502.

    Article  Google Scholar 

  20. Safavi A, Lin Y, Skarsgard ED . Canadian Pediatric Surgical Network. Perinatal management of congenital diaphragmatic hernia: when and how should babies be delivered? Results from the Canadian Pediatric Surgery Network. J Pediatr Surg 2010; 45 (12): 2334–2339.

    Article  Google Scholar 

  21. CDH Study Group: Morini F, Valfre L, Capolupo I, Lally KP, Lally PA et al. Congenital diaphragmatic hernia: defect size correlates with developmental defect. J Pediatr Surg 2013; 48 (6): 1177–1182.

    Article  Google Scholar 

Download references

Acknowledgements

The CHNC (http://www.thechnc.org) has partnered with Children’s Hospital Association (Overland Park, KS, USA and Alexandria, VA, USA) to design, launch and maintain the CHND. We are indebted to the following institutions that serve infants and their families, and these institutions also have invested in and continue to participate in the CHND. For more information about CHND, please contact chnd@childrenshospitals.org. We also thank the site sponsors, data abstractors, and staff for the CHND for their investments in this program. The CHND site sponsors are:

1. Children's Healthcare of Atlanta at Egleston, Atlanta, GA (Francine Dykes, Anthony Piazza)

2. Children’s Healthcare of Atlanta at Scottish Rite, Atlanta, GA (Gregory Sysyn)

3. Children's of Alabama, Birmingham, AL (Carl Coghill)

4. Le Bonheur Children's Hospital, Memphis, TN (Ramasubbareddy Dhanireddy)

5. Boston Children’s Hospital, Boston, MA (Anne Hansen)

6. Ann and Robert H Lurie Children’s Hospital of Chicago, Chicago, IL (Karna Murthy)

7. Nationwide Children's Hospital, Columbus, OH (Kristina Reber)

8. Children's Medical Center Dallas, Dallas, TX (Rashmin Savani)

9. Children's Hospital Colorado, Aurora, CO (Theresa Grover)

10. Children’s Hospital of Michigan, Detroit, MI (Girija Natarajan)

11. Cook Children's Medical Center, Fort Worth, TX (Jonathan Nedrelow, Annie Chi)

12. Texas Children's Hospital, Houston, TX (Stephen Welty)

13. Children's Mercy-Kansas City, MO (Eugenia Pallotto)

14. Arkansas Children's Hospital, Little Rock, AR (Becky Rodgers, Robert Lyle)

15. Children’s Hospital Los Angeles, Los Angeles, CA (Lisa Kelly*, Steven Chin)

16. UCSF Benioff Children's Hospital Oakland, Oakland, CA (David Durand, Jeanette Asselin, Priscilla Joe)

17. The Children's Hospital of Philadelphia, Philadelphia, PA (Jacquelyn Evans, Michael Padula)

18. Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA (Beverly Brozanski)

19. St Louis Children's Hospital, St Louis, MO (Joan Rosenbaum, Tasmin Najaf, Amit Mathur, Rakesh Rao)

20. All Children's Hospital, St Petersburg, FL (Victor McKay)

21. Rady Children's Hospital San Diego, San Diego, CA (Mark Speziale)

22. Children's National Medical Center, Washington, DC (Billie Short)

23. Alfred I.duPont Hospital for Children, Wilmington, DE (Kevin Sullivan)

24. Primary Children’s Hospital, Salt Lake City, UT (Robert DiGeronimo)

25. Children’s Hospital of Wisconsin, Milwaukee, WI (Michael Uhing)

26. Children’s Hospital and Medical Center, Omaha, NE (Lynne Willett, John Grebe)

27. Florida Hospital for Children, Orlando, FL (Rajan Wadhawan)

28. Seattle Children’s, Seattle, WA (Elizabeth Jacobson Misbe)

* Deceased.

Author contributions

Each author participated and contributed to the design (KM, EKP, JG, BSB, NFMP, IZ, SK, LC, NR, TRG), data collection (KM, JMA, BLS, DJD, EKP, MAP, FDD, KMR, JRE), analysis (all) and/or interpretation (all). Also, all authors drafted and/or revised the manuscript critically for substantive content. Each reviewed and approved this submitted version. Dr Murthy wrote the first draft of this paper.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to K Murthy.

Ethics declarations

Competing interests

The authors declare no conflict of interest.

Additional information

Supplementary Information accompanies the paper on the Journal of Perinatology website

Supplementary information

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Murthy, K., Pallotto, E., Gien, J. et al. Predicting death or extended length of stay in infants with congenital diaphragmatic hernia. J Perinatol 36, 654–659 (2016). https://doi.org/10.1038/jp.2016.26

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/jp.2016.26

This article is cited by

Search

Quick links