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

Affiliations
  • 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. kimhans@snu.ac.kr
  • 4Department of Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.

Abstract

PURPOSE
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.
METHODS
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.
RESULTS
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.
CONCLUSION
Using the RSS as a prognostic predictor with simple calculations will help clinicians plan CDH management.

Keyword

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

MeSH Terms

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

Figure

  • 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.


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