Neonatal Med.  2018 Aug;25(3):102-108. 10.5385/nm.2018.25.3.102.

Respiratory Severity Score as a Predictive Factor for the Mortality of Congenital Diaphragmatic Hernia

  • 1Department of Pediatrics, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea.
  • 2Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
  • 3Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
  • 4Department of Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.


Congenital diaphragmatic hernia (CDH) is rare but potentially fatal. The overall outcome is highly variable. This study aimed to identify a simple and dynamic parameter that helps predict the mortality of CDH patients in real time, without invasive tests.
We conducted a retrospective chart review of 59 CDH cases. Maternal and fetal information included the gestational age at diagnosis, site of defect, presence of liver herniation, and lung-to-head ratio (LHR) at 20 to 29 weeks of gestational age. Information regarding postnatal treatment, including the number of days until surgery, the need for inhaled nitric oxide (iNO), the need for extracorporeal membrane oxygenation (ECMO), and survival, was collected. The highest respiratory severity score (RSS) within 24 hours after birth was also calculated.
Statistical analysis showed that a younger gestational age at the initial diagnosis (P < 0.001), a lower LHR (P=0.001), and the presence of liver herniation (P=0.003) were prenatal risk factors for CDH mortality. The RSS and use of iNO and ECMO were significant factors affecting survival. In the multivariate analysis, the only remaining significant risk factor was the highest preoperative RSS within 24 hours after birth (P=0.002). The area under the receiver operating characteristic curve was 0.9375, with a sensitivity of 91.67% and specificity of 83.87% at the RSS cut-off value of 5.2. The positive and negative predictive values were 82.14% and 92.86%, respectively.
Using the RSS as a prognostic predictor with simple calculations will help clinicians plan CDH management.


Respiratory severity score; Congenital diaphragmatic hernia; Predictive factor; Mortality; Prognosis; Neonate

MeSH Terms

Extracorporeal Membrane Oxygenation
Gestational Age
Hernias, Diaphragmatic, Congenital*
Infant, Newborn
Multivariate Analysis
Nitric Oxide
Retrospective Studies
Risk Factors
ROC Curve
Sensitivity and Specificity
Nitric Oxide


  • Figure 1. Flow chart of disease course in patients admitted between December 2006 and June 2015. Abbreviations: CDH, congenital diaphragmatic hernia; ECMO, extracorporeal membrane oxygenation.

  • Figure 2. Receiver operating characteristic (ROC) curve of the highest pre-operative respiratory severity score within 24 hours after birth.


1. Barriere F, Michel F, Loundou AD, Fouquet V, Kermorvant E, Blanc S, et al. One-year outcome for congenital diaphragmatic hernia: results from the French National Register. J Pediatr. 2018; 193:204–10.
2. Brindle ME, Cook EF, Tibboel D, Lally PA, Lally KP; Congenital Diaphragmatic Hernia Study Group. A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns. Pediatrics. 2014; 134:e413–9.
3. Schultz CM, DiGeronimo RJ, Yoder BA; Congenital Diaphragmatic Hernia Study Group. Congenital diaphragmatic hernia: a simplified postnatal predictor of outcome. J Pediatr Surg. 2007; 42:510–6.
4. van den Hout L, Reiss I, Felix JF, Hop WC, Lally PA, Lally KP, et al. Risk factors for chronic lung disease and mortality in newborns with congenital diaphragmatic hernia. Neonatology. 2010; 98:370–80.
5. 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.
6. Baird R, MacNab YC, Skarsgard ED; Canadian Pediatric Surgery Network. Mortality prediction in congenital diaphragmatic hernia. J Pediatr Surg. 2008; 43:783–7.
7. Merrill JD, Ballard RA, Cnaan A, Hibbs AM, Godinez RI, Godinez MH, et al. Dysfunction of pulmonary surfactant in chronically ventilated premature infants. Pediatr Res. 2004; 56:918–26.
8. Katz LA, Klein JM. Repeat surfactant therapy for postsurfactant slump. J Perinatol. 2006; 26:414–22.
9. Malkar MB, Gardner WP, Mandy GT, Stenger MR, Nelin LD, Shepherd EG, et al. Respiratory severity score on day of life 30 is predictive of mortality and the length of mechanical ventilation in premature infants with protracted ventilation. Pediatr Pulmonol. 2015; 50:363–9.
10. Ballard RA, Truog WE, Cnaan A, Martin RJ, Ballard PL, Merrill JD, et al. Inhaled nitric oxide in preterm infants undergoing mechanical ventilation. N Engl J Med. 2006; 355:343–53.
11. Iyer NP, Mhanna MJ. Non-invasively derived respiratory severity score and oxygenation index in ventilated newborn infants. Pediatr Pulmonol. 2013; 48:364–9.
12. Grushka JR, Laberge JM, Puligandla P, Skarsgard ED; Canadian Pediatric Surgery Network. Effect of hospital case volume on outcome in congenital diaphragmatic hernia: the experience of the Canadian Pediatric Surgery Network. J Pediatr Surg. 2009; 44:873–6.
13. Weems MF, Jancelewicz T, Sandhu HS. Congenital diaphragmatic hernia: maximizing survival. NeoReviews. 2016; 17:e705. –18.
14. Kim SY, Shin SH, Kim HS, Jung YH, Kim EK, Choi JH. Pulmonary arterial hypertension after ibuprofen treatment for patent ductus arteriosus in very low birth weight infants. J Pediatr. 2016; 179:49–53. e1.
15. Lusk LA, Wai KC, Moon-Grady AJ, Steurer MA, Keller RL. Persistence of pulmonary hypertension by echocardiography predicts short-term outcomes in congenital diaphragmatic hernia. J Pediatr. 2015; 166:251–6. e1.
16. Keller RL, Tacy TA, Hendricks-Munoz K, Xu J, Moon-Grady AJ, Neuhaus J, et al. Congenital diaphragmatic hernia: endothelin-1, pulmonary hypertension, and disease severity. Am J Respir Crit Care Med. 2010; 182:555–61.
17. Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, et al. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015; 132:2037–99.
18. Snoek KG, Reiss IK, 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.
19. McHoney M, Hammond P. Role of ECMO in congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal Ed. 2018; 103:F178–81.
20. Fletcher K, Chapman R, Keene S. An overview of medical ECMO for neonates. Semin Perinatol. 2018; 42:68–79.
21. Hollinger LE, Lally PA, Tsao K, Wray CJ, Lally KP; Congenital Diaphragmatic Hernia Study Group. A risk-stratified analysis of delayed congenital diaphragmatic hernia repair: does timing of operation matter? Surgery. 2014; 156:475–82.
22. Kamata S, Usui N, Ishikawa S, Okuyama H, Kitayama Y, Sawai T, et al. Prolonged preoperative stabilization using high-frequency oscillatory ventilation does not improve the outcome in neonates with congenital diaphragmatic hernia. Pediatr Surg Int. 1998; 13:542–6.
23. Glenski JA, Marsh HM, Hall RT. Calculation of mean airway pressure during mechanical ventilation in neonates. Crit Care Med. 1984; 12:642–4.
24. Green C, Yohannan MD. Umbilical arterial and venous catheters: placement, use, and complications. Neonatal Netw. 1998; 17:23–8.
25. Huning BM, Horsch S, Roll C. Blood sampling via umbilical vein catheters decreases cerebral oxygenation and blood volume in preterm infants. Acta Paediatr. 2007; 96:1617–21.
26. Del Vecchio A, Franco C, Petrillo F, D'Amato G. Neonatal transfusion practice: when do neonates need red blood cells or platelets? Am J Perinatol. 2016; 33:1079–84.
27. Mhanna MJ, Iyer NP, Piraino S, Jain M. Respiratory severity score and extubation readiness in very low birth weight infants. Pediatr Neonatol. 2017; 58:523–8.
28. DiBlasi RM, Myers TR, Hess DR. Evidence-based clinical practice guideline: inhaled nitric oxide for neonates with acute hypoxic respiratory failure. Respir Care. 2010; 55:1717–45.
29. Campbell BT, Herbst KW, Briden KE, Neff S, Ruscher KA, Hagadorn JI. Inhaled nitric oxide use in neonates with congenital diaphragmatic hernia. Pediatrics. 2014; 134:e420. –6.
30. The Neonatal Inhaled Nitric Oxide Study Group (NINOS). Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. Pediatrics. 1997; 99:838–45.
31. Putnam LR, Tsao K, Morini F, Lally PA, Miller CC, Lally KP, et al. Evaluation of variability in inhaled nitric oxide use and pulmonary hypertension in patients with congenital diaphragmatic hernia. JAMA Pediatr. 2016; 170:1188–94.
32. Ijsselstijn H, van Heijst AF. Long-term outcome of children treated with neonatal extracorporeal membrane oxygenation: increasing problems with increasing age. Semin Perinatol. 2014; 38:114–21.
33. Inamura N, Usui N, Okuyama H, Nagata K, Kanamori Y, Fujino Y, et al. Extracorporeal membrane oxygenation for congenital diaphragmatic hernia in Japan. Pediatr Int. 2015; 57:682–6.
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