Original article
Long-term neurodevelopmental outcomes of congenital diaphragmatic hernia survivors not treated with extracorporeal membrane oxygenation

https://doi.org/10.1016/j.jpedsurg.2010.12.023Get rights and content

Abstract

Background/Purpose

Although there has been a marked improvement in the survival of children with congenital diaphragmatic hernia (CDH) in the past 2 decades, there are few reports of long-term neurodevelopmental outcome in this population. The present study examined neurodevelopmental outcomes in 10- to 16-year-old CDH survivors not treated with extracorporeal membrane oxygenation (ECMO).

Methods

Parents of 27 CDH survivors completed questionnaires assessing medical problems, daily living skills, educational outcomes, behavioral problems, and executive functioning. Fifteen CDH survivors and matched full-term controls completed standardized intelligence, academic achievement, phonological processing, and working memory tests.

Results

Non–ECMO-treated CDH survivors demonstrated high rates of clinically significant difficulties on standardized academic achievement measures, and 14 of the 27 survivors had a formal diagnosis of specific learning disability, attention deficit hyperactivity disorder, or developmental disability. Specific problems with executive function, cognitive and attentional weaknesses, and social difficulties were more common in CDH patients than controls. Perioperative hypocapnia was linked to executive dysfunction, behavioral problems, lowered intelligence, and poor achievement in mathematics.

Conclusions

Non–ECMO-treated CDH survivors are at substantial risk for neurodevelopmental problems in late childhood and adolescence.

Section snippets

Participants

A cohort of 56, 10- to 16-year-old, CDH survivors was identified from the medical records at the Hospital for Sick Children in Toronto, ON, Canada; this represented the entire cohort of survivors born during a 6-year period. At the time these individuals were born, our institution used high-frequency oscillating ventilation as a rescue therapy [13]. Although ECMO was available, it was considered a treatment of last resort restricted to babies who were not expected to survive without such

Medical histories

To ascertain whether the participating CDH survivors were representative of our entire cohort of survivors, we extracted information from the medical records for all survivors regarding: gestational age; Apgar scores (1 and 5 minutes); side/size of hernia; need for surgical patch; highest preoperative and postoperative pH and CO2; length of time/type of ventilation; use of alkalinization therapy, steroids, and muscle paralysis agents; presence/severity of chronic lung disease; duration of

Discussion

Short-term neurodevelopmental outcomes of non–ECMO-treated CDH survivors are considered to be quite good, with low rates of developmental issues being reported in preschoolers [4], [6], [10]. As CDH survivors move through their school-age years, however, neurodevelopmental weaknesses become more apparent. In our study, between 23% and 46% of non–ECMO-treated CDH survivors tested in late childhood and adolescence demonstrated clinically significant academic difficulties on standardized

Conclusion

The findings described here may prove useful in guiding prospective research with CDH patients. They also contribute to our understanding of the effects of hypocapnia or hyperventilation on the developing brain and highlight the need to follow older cohorts of CDH survivors who appear to be at substantial risk for neurodevelopmental problems. Health care professionals need to be aware of these problems so that they can counsel families antenatally and help parents plan prospectively for their

Acknowledgment

We would like to thank the families who participated in this research.

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      Moreover, 6% (range 2%–9%; n = 9) incidence of strabismus was found in CDH survivors, as described in four studies (n = 141) [5,21,52,88]. Six studies evaluated behavioral problems in children born with CDH (n = 167) and reported significant impairment in 20% (range 8%–50%, n = 33) of them [29,41,47,48,80,84]. Four studies (n = 122) reported a 19% (range 7%–39%, n = 23) incidence of attention deficit in CDH survivors [41,48,80,88].

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    This research was supported by a grant from the Natural Sciences and Engineering Council of Canada to L.S.J. D.T. was supported by the Swiss National Foundation and the Margarete and Walter Lichtenstein Foundation. The authors have no competing interests. D.T. is now with the Division of Pediatric Intensive Care and Pulmonology, University Children's Hospital of Basel, Basel, Switzerland. V.F. is now a consulting psychologist with Central West Region and Durham Infant and Child Development Services.

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