pn Perinatology Perinatology 2508-4887 2508-4895 The Korean Society of Perinatology 10.14734/PN.2018.29.02.72 pn-29-72 Original Article Trends in Treatment Outcome and Critical Predictors of Mortality for Congenital Diaphragmatic Hernia in a Single Center HwangJi HyeMD 1 KimChae YoungMD 1 ParkHye WonMD, PhD 2 NamgoongJung-ManMD 3 KimDae YeonMD, PhD 3 KimSeong-ChulMD, PhD 3 LeeMi YoungMD, PhD 4 WonHye SungMD, PhD 4 ShimJae YoonMD, PhD 4 LeePil RyangMD, PhD 4 KimAhmMD, PhD 4 JungEuiseokMD 1 LeeByong SopMD, PhD 1 KimKi-SooMD, PhD 1 KimEllen Ai-RhanMD, PhD 1 Division of Neonatalogy, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul Division of Pediatric Surgery, Department of Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul; Department of Obstetrics and Gynecology, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea Correspondence to Ellen Ai-Rhan Kim, MD, PhD Division of Neonatology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea Tel: +82-2-3010-3377 Fax: +82-2-3010-6978 E-mail: arkim@amc.seoul.kr Dae Yeon Kim, MD, PhD Division of Pediatric Surgery, Department of Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea Tel: +82-2-3010-3961 Fax: +82-2-3010-6863 E-mail: kimdy@amc.seoul.kr 062018 19062018 29272 77 06122017 31012018 20022018 Copyright © 2018 The Korean Society of Perinatology 2018 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Objective

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).

 Methods

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.

 Results

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%.

 Conclusion

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.

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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.

(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).

(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.

(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.

Patient Demographics between Survivors and Non-survivors

  Total Survivors Non-survivors P-value
Prenatal factors        
Prenatal diagnosis 219/264 (83.0) 147/185 (79.5) 72/79 (91.1) 0.021
Polyhydramnios 50/227 (22.0) 25/154 (16.2) 25/73 (34.2) 0.002
Associated major congenital anomalies 19/264 (7.2) 3/185 (1.6) 16/79 (20.3) 0.000
O/E LHR (%) 54.8±18.3 62.0±15.7 38.5±12.3 0.000
Neonatal factors        
Male 154/264 (58.3) 108/185 (58.4) 46/79 (58.2) 0.982
Gestational age (wks) 38.0±2.2 38.4±1.3 37.1±3.2 0.036
Birth weight (g) 2,934.2±578.2 3,030.3±460.8 2,709±743.8 0.005
Cesarean section 146/264 (55.3) 93/185 (50.3) 53/79 (67.1) 0.012
1-min Apgar score 5.5±1.9 6.0±1.6 4.3±1.9 0.000
5-min Apgar score 7.4±1.5 7.9±1.1 6.3±1.7 0.000
Left CDH 226/264 (85.6) 162/185 (87.6) 64/79 (81.0) 0.165
Inborn 230/264 (87.1) 155/185 (83.8) 75/79 (94.9) 0.013
Surgical factors        
Defect size (cm) 4.4±1.6 4.3±1.5 5.0±0.0 <0.001
Need for patch 47/222 (21.2) 30/185 (16.2) 17/79 (45.9) <0.001
Presence of hernia sac 66/205 (32.2) 58/177 (32.8) 8/28 (28.6) 0.659
Number of herniated organ 3.1±1.2 3.0±1.2 3.6±1.3 0.002

Values are presented as mean±standard deviation or number (%). Abbreviations: O/E LHR, observed/expected lung-to-head ratio; CDH, congenital diaphragmatic hernia.