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

Ji Hye Hwang; Chae Young Kim; Hye Won Park; Jung-Man Namgoong; Dae Yeon Kim; Seong-Chul Kim; Mi Young Lee; Hye Sung Won; Jae Yoon Shim; Pil Ryang Lee; Ahm Kim; Euiseok Jung; Byong Sop Lee; Ki-Soo Kim; Ellen Ai-Rhan Kim

doi:10.14734/PN.2018.29.02.72

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Hwang, Kim, Park, Namgoong, Kim, Kim, Lee, Won, Shim, Lee, Kim, Jung, Lee, Kim, and Kim: Trends in Treatment Outcome and Critical Predictors of Mortality for Congenital Diaphragmatic Hernia in a Single Center

Original Article

Perinatology 2018;29(2):72-77.

Published online: 19 June 2018

DOI: https://doi.org/10.14734/PN.2018.29.02.72

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

Ji Hye Hwang, MD¹, Chae Young Kim, MD¹, Hye Won Park, MD, PhD², Jung-Man Namgoong, MD³, Dae Yeon Kim, MD, PhD³, Seong-Chul Kim, MD, PhD³, Mi Young Lee, MD, PhD⁴, Hye Sung Won, MD, PhD⁴, Jae Yoon Shim, MD, PhD⁴, Pil Ryang Lee, MD, PhD⁴, Ahm Kim, MD, PhD⁴, Euiseok Jung, MD¹, Byong Sop Lee, MD, PhD¹, Ki-Soo Kim, MD, PhD¹, Ellen Ai-Rhan Kim, MD, PhD¹

¹Division of Neonatalogy, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul

²Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul

³Division of Pediatric Surgery, Department of Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul;

⁴Department 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

Received 6 December 201731 January 2018 Accepted 20 February 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.

Abstract

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.

Keywords: Congenital diaphragmatic hernia, Prenatal diagnosis, Mortality

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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.

Table 1.

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.

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