Perinatology.  2018 Jun;29(2):72-77. 10.14734/PN.2018.29.2.72.

Trends in Treatment Outcome and Critical Predictors of Mortality for Congenital Diaphragmatic Hernia in a Single Center

  • 1Division of Neonatalogy, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
  • 2Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Korea.
  • 3Division of Pediatric Surgery, Department of Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
  • 4Department of Obstetrics and Gynecology, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.


To review trends in treatment outcomes, to determine risk factors for mortality, and to present a cut-off values of observed/expected lung to head ratio (O/E LHR) predicting mortality in infants with congenital diaphragmatic hernia (CDH).
A retrospective study of infants with CDH treated in the neonatal intensive care unit at tertiary medical center over 27 years (1989 to 2016) was done. We analyzed the factors associated with mortality and based on significant factors in predicting the mortality, the best cut-off value of O/E LHR was sought.
A total of 264 patients were included for the study. The mean gestational age and birth weight were 38.0±2.2 weeks and 2,934.2±578.2 g, respectively. Seventy nine patients (29.9%) died. The range of yearly mortality rate varied from 0 to 67%. Prenatal, neonatal, and surgical factors associated to mortality included time of prenatal diagnosis, polyhydramnios, O/E LHR, associated major congenital anomalies (cardiac, gastrointestinal or chromosomal anomalies), gestational age, birth weight, Apgar scores, initial pH and pCO2, and number of herniated organs. Among 62 fetuses whose O/E LHR were available, O/E LHR and major congenital anomalies were independent risk factors for mortality (area under curve=0.918), fetuses with O/E LHR < 44% without congenital anomalies showed predicted mortality of 72% and fetuses with O/E LHR ≥44% without congenital anomalies showed expected mortality of 7%.
The mortality of Infants with CDH has not improved despite advance of neonatal care. The O/E LHR and the presence of major congenital anomalies could be used adjunctively when counseling parents about the prognosis.


Congenital diaphragmatic hernia; Prenatal diagnosis; Mortality

MeSH Terms

Birth Weight
Gestational Age
Hernias, Diaphragmatic, Congenital*
Hydrogen-Ion Concentration
Infant, Newborn
Intensive Care, Neonatal
Prenatal Diagnosis
Retrospective Studies
Risk Factors
Treatment Outcome*


  • Fig. 1 The mortality rate by year was from 0 to 67% and did not improve significantly despite the progress in treatment modalities. CDH, congenital diaphragmatic hernia.

  • Fig. 2 (A) Period was divided into the introduction of inhaled nitric oxide and high frequency oscillation ventilation and the introduction of extracorporeal membrane oxygenation. The mortality rates of each period were 40.0%, 21.6%, and 32.4%, respectively (P=0.144). (B) The mortality rate of delayed operation was significantly lower than early operation (P=0.003).

  • Fig. 3 (A) In the absence of O/E LHR value, 5-minute Apgar score, first postnatal pH, and associated major congenital anomalies were important to predict mortality (AUC=0.883). (B) In the presence of the O/E LHR value, O/E LHR and associated major congenital anomalies were more significant to predict mortality (AUC=0.918). O/E LHR, observed/expected lung-to-head ratio; AUC, area under the curve.

  • Fig. 4 (A) Youden index of O/E LHR is 44% using receiver operating characteristic (ROC) curve. (B) The 44% of O/E LHR shows 79% sensitivity and 88% specificity. O/E LHR, observed/expected lung-to-head ratio.


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