Original Article
Developmental outcomes of children with congenital diaphragmatic hernia: A multicenter prospective study,☆☆,☆☆☆,,★★

https://doi.org/10.1016/j.jpedsurg.2013.02.041Get rights and content

Abstract

Purpose

To determine developmental outcomes and associated factors in patients with congenital diaphragmatic hernia (CDH) at 2 years of age.

Methods

This is a multicenter prospective study of a CDH birth cohort. Clinical and socioeconomic data were collected. Bayley Scales of Infant Development (BSID-III) and Vineland Adaptive Behavior Scales (VABS-II) were performed at 2 years of age.

Results

BSID-III and VABS-II assessments were completed on 48 and 49 children, respectively. The BSID-III mean cognitive, language, and motor scores were significantly below the norm mean with average scores of 93 ± 15, 95 ± 16, and 95 ± 11. Ten percent (5/47) scored more than 2 standard deviations below the norm on one or more domains. VABS-II scores were similar to BSID-III scores with mean communication, daily living skills, social, motor, adaptive behavior scores of 97 ± 14, 94 ± 16, 93 ± 13, 97 ± 10, and 94 ± 14. For the BSID-III, supplemental oxygen at 28 days, a prenatal diagnosis, need for extracorporeal membrane oxygenation (ECMO) and exclusive tube feeds at time of discharge were associated with lower scores. At 2 years of age, history of hospital readmission and need for tube feeds were associated with lower scores. Lower socioeconomic status correlated with lower developmental scores when adjusted for significant health factors.

Conclusion

CDH patients on average have lower developmental scores at 2 years of age compared to the norm. A need for ECMO, oxygen at 28 days of life, ongoing health issues and lower socioeconomic status are factors associated with developmental delays.

Section snippets

Cohort

Subjects were recruited as part of the DHREAMS study (Diaphragmatic Hernia Research & Exploration, Advancing Molecular Science; http://www.cdhgenetics.com). The DHREAMS study is a multicenter prospective cohort of neonates with a diaphragm anomaly [17]. Each participating center is a regional tertiary care hospital with a neonatal intensive care unit that includes an extracorporeal membrane oxygenation (ECMO) program. Eligibility criteria are that the infant has a radiologically confirmed

Patient population

A total of 68 (69%) of the 99 eligible infants consented to enrollment in the DHREAMS study. Those eligible who did not enroll either declined participation or the child died before the family could be approached to discuss the study. Of those enrolled, 14 (21%) children died prior to discharge and one died post discharge prior to 2 years of age. Of the 53 survivors, the families of 2 children declined the 2-year assessment and 2 were unavailable. English was the dominant language for all

Discussion

In the era of prenatal diagnosis and counseling, accurate understanding of the long-term developmental outcomes in children with CDH is critical. The modern surgical and respiratory management of infants affected with CDH has improved the current survival rate to approximately 70%–90% [1], [26], [27], [28]. As survival has improved, many reports have focused on the neonatal and long-term morbidities [18], [29], [30]. Few studies have prospectively addressed developmental outcomes in CDH

Limitations

Limitations of our study include incomplete enrollment, some loss to follow-up, and modest size of our cohort. There was a significant number of patients who were eligible for the study but died before they could be invited to the study or declined enrollment. Because these patients were not enrolled, we could not collect outcome data on them. It is possible that the mortality, morbidity and/or SES were different in this group compared with our study population, and this may bias our results.

Conclusion

We report the developmental outcomes and associated factors on a large multicenter prospective CDH birth cohort. Our study is strengthened by the use of the same standardized developmental assessment, completion of the assessments at a single age, and a high retention rate. As survival rates continue to improve, our results highlight the importance of monitoring CDH patients for developmental problems. CDH patients are at risk for developmental delays with scores significantly below the norm

Acknowledgments

We greatly appreciate the families who participated in this study and all the clinical care teams who assisted with study coordination. We thank Jeannie Kreutzman, and Robert Drongowski from University of Michigan; Trish Burns from Cincinnati Children's Hospital Medical Center; Sheila Horak from University of Nebraska; and Laurie Luther from University of Pittsburgh. We also thank Lan Yu for completing the genetic analysis.

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    Funding: This work was supported by NIH grant HD057036. The funder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

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    Financial disclosure: The authors have no financial relationships relevant to this article to disclose.

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    Conflict of interest: The authors have no conflicts of interest to disclose.

    Clinical trials registry: Diaphragmatic Hernia Research & Exploration, Advancing Molecular Science (DHREAMS), NCT00950118.

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    Contributors' statement: Dr. Farkouh conceptualized and designed the study, collected data, drafted the manuscript, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Chung conceptualized and designed the study, drafted the manuscript, and approved the final manuscript as submitted. Dr. Arkovitz conceptualized and designed the study, drafted the manuscript, and approved the final manuscript as submitted. Dr. Aspelund collected data, drafted the manuscript, critically reviewed the manuscript, and approved the final manuscript as submitted. Ms. Wynn coordinated and supervised data collection, drafted the manuscript, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Zygmunt completed the developmental assessments, critically reviewed the manuscript, and approved the final manuscript as submitted. Ms. Gratton completed the developmental assessments, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Jackson completed the developmental assessments, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Butcher completed the developmental assessments, critically reviewed the manuscript, and approved the final manuscript as submitted. Ms. Brennan completed the developmental assessments, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Stolar collected data, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Mychaliska collected data, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Lim collected data, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Potoka collected data, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Azarow collected data, critically reviewed the manuscript, and approved the final manuscript as submitted. Dr. Crombleholme collected data, critically reviewed the manuscript, and approved the final manuscript as submitted. Ms. Zhang completed the statistical analysis, critically reviewed the manuscript, and approved the final manuscript as submitted. Mr. Duong completed the statistical analysis, critically reviewed the manuscript, and approved the final manuscript as submitted.

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