Abstract
Objective
This case study examined the neurodevelopmental profile associated with congenital diaphragmatic hernia (CDH). CDH is primarily linked to chromosomal abnormality and results in pulmonary hypoplasia and significant respiratory difficulties. CDH occurs in 1 in 2,500 live births in the United States. More severe cases require extracorporeal membrane oxygenation (ECMO) and surgical hernia repair. Survivors are at risk of pulmonary hypertension, gastrointestinal disorders, structural brain abnormalities, and neurodevelopmental disorders. Past studies suggested early ECMO and surgical repair are associated with greater subsequent impairment.
Method
A 16-year-old male with a history of CDH presented for neuropsychological evaluation. History is remarkable for ECMO, surgical repair, pulmonary hypertension, and gastrostomy tube.
Results
Assessment revealed significant neurodevelopmental deficits impacting multiple domains including cognitive reasoning, visual construction, verbal memory, and executive functioning. Decoding and underlying phonemic awareness were preserved, while reading comprehension and applied math were significantly impaired, suggesting significant weaknesses in higher-order language. Social intent was strong despite weak social cognition. Elevated symptoms of anxiety and depression were evident.
Conclusions
CDH carries significant morbidity secondary to compromised respiratory functions and long-term pulmonary hypertension. Neurodevelopmental deficits are strongly associated with CDH and merit early detection, intervention, and follow-up. This case study illustrates the global neurodevelopmental difficulties in CDH requiring ECMO and surgical repair. This study illustrates escalating difficulties navigating academic and social demands as the need for higher order language and social cognition increase through adolescence. Serial neuropsychological assessments may be useful in facilitating and adapting interventions, assessing progress, detecting behavioral health symptoms, and contributing to optimal outcomes in CDH.
1762 CONGENITAL DIAPHRAGMATIC HERNIA- CONTRIBUTION OF PULMONARY ARTERIAL REMODELLING TO PERSISTENT PULMONARY HYPERTENSION
