The current profile and outcome of congenital diaphragmatic hernia: A nationwide survey in Japan

Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582, Japan. Electronic address: .
Journal of Pediatric Surgery (Impact Factor: 1.39). 04/2013; 48(4):738-44. DOI: 10.1016/j.jpedsurg.2012.12.017
Source: PubMed


Few nationwide surveys have been reported regarding the perinatal status, clinical course and postnatal outcome of cases with congenital diaphragmatic hernia (CDH). The aim of this study was to review the current profile and the outcomes of a large cohort of CDH cases in Japan.
A nationwide retrospective cohort study was conducted on neonates diagnosed to have CDH between January 2006 and December 2010. The questionnaires were sent to 159 representative regional institutions and 109 (68.6%) institutions responded to the preliminary survey which had 674 cases. Eleven institutions which had 60 CDH neonates did not respond to the second questionnaire, and 26 institutions had no cases. Finally, 614 CDH neonates from 72 institutions had been collected and were used in the detailed survey. The perinatal status, clinical course and the postnatal outcome were reviewed. Survival was defined as infants alive at hospital discharge, at the time of transfer or still in the hospital at the time of questionnaire, which was confirmed during the period from July 2011 to November 2011 by the investigators.
Four hundred sixty-three (75.4%) of 614 CDH neonates survived in this study. The overall survival rate of neonates with isolated CDH was 84.0%. A total of 444 (72.0%) patients were prenatally diagnosed, and had a survival rate of 70.8%. Four hundred thirty-three (70.9%) patients were treated with high-frequency oscillatory ventilation (HFOV) as the initial ventilation, 344 (56.0%) patients received inhaled nitric oxide (iNO) and 43 (7.0%) required extracorporeal membrane oxygenation (ECMO). The overall survival rates of the CDH neonates who had been treated using HFOV, iNO and ECMO were 74.3%, 68.3% and 37.2%, respectively.
This study demonstrated that the current status for CDH treatment in Japan and the overall survival rate were comparable to those of recent reports from other countries.

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    ABSTRACT: Background: Congenital diaphragmatic hernia is associated with cardiovascular malformation. Many prognostic factors have been identified for isolated congenital diaphragmatic hernia; however, reports of concurrent congenital diaphragmatic hernia and cardiovascular malformation in infants are limited. This study evaluated congenital diaphragmatic hernia associated with cardiovascular malformation in infants. Factors associated with prognosis for patients were also identified. Methods: This retrospective cohort study was based on a Japanese survey of congenital diaphragmatic hernia patients between 2006 and 2010. Frequency and outcome of cardiovascular malformation among infants with congenital diaphragmatic hernia were examined. Severity of congenital diaphragmatic hernia and cardiovascular malformation were compared as predictors of mortality and morbidity. Results: Cardiovascular malformation was identified in 76 (12.3%) of 614 infants with congenital diaphragmatic hernia. Mild cardiovascular malformation was detected in 19 (33.9%) and severe cardiovascular malformation in 37 (66.1%). Their overall survival rate at discharge was 46.4%, and the survival rate without morbidity was 23.2%. Mortality and morbidity at discharge were more strongly associated with severity of cardiovascular malformation (adjusted OR 7.69, 95%CI 1.96-30.27; adjusted OR 7.93, 95%CI 1.76-35.79, respectively) than with severity of congenital diaphragmatic hernia. Conclusions: The prognosis for infants with both congenital diaphragmatic hernia and cardiovascular malformation remains poor. Severity of cardiovascular malformation is a more important predictive factor for mortality and morbidity than severity of congenital diaphragmatic hernia.
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    ABSTRACT: Abstract Objectives: To evaluate the incidence, risk factors and neonatal outcomes associated with a congenital diaphragmatic hernia (CDH). Study Design: We conducted a population-based cohort study using the CDC's Linked Birth-Infant Death and Fetal Death data files on all births and fetal deaths in the United States between 1995 and 2002. We estimated the yearly incidence of CDH and measured its adjusted effect on various outcomes using unconditional logistic regression analysis. Results: 32,145,448 births during the 8-year study period met the study's inclusion criteria. The incidence of CDH was 1.93/10,000 births. Risk factors for the development of CDH included fetal male gender [OR 1.12, 95% CI: 1.06, 1.17], maternal age beyond 40 [OR 1.51, 95% CI: 1.26, 1.80], Caucasian ethnicity [OR 1.15, 95% CI: 1.10, 1.21], smoking [OR 1.34, 95% CI: 1.22, 1.46] and alcohol use during pregnancy [OR 1.37, 95% CI: 1.05, 1.79]. As compared to fetuses with no CDH, fetuses with CDH were at an increased risk of preterm birth [OR 2.90, 95% CI: 2.72, 3.11], IUGR [OR 3.84, 95% CI: 3.51, 4.18], stillbirth [OR 9.65, 95% CI: 8.20, 11.37], and overall infant death [OR: 94.80, 95% CI: 88.78, 101.23]. The 1-year mortality was 45.89%. Conclusion: Congenital diaphragmatic hernia is strongly associated with an increased risk of adverse pregnancy, fetal and neonatal outcomes. These findings may be helpful in counselling pregnancies affected by CDH, and may aid in the understanding of the burden of this condition at the public health level.
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