(815) Abnormal neurodevelopment in CDH survivors is not dependent on severity or fetal surgery

Children with congenital diaphragmatic hernia (CDH) are at increased risk for neurodevelopmental delay. Fetoscopic Endoluminal Tracheal Occlusion(FETO) increases survival in left-sided CDH fetuses with severe pulmonary hypoplasia. To our knowledge there is no data on the neurologic outcome comparing children with prenatally diagnosed CDH treated either ante- or postnatal.

Study Design:

This is a multicenter prospective cohort study performed in 4 experienced fetal treatment centers (Barcelona, Spain; São Paulo, Brazil; Leuven, Belgium; London, UK). Parents of children, diagnosed in utero with isolated left-sided CDH, and aged between  1 and 6 years at the moment of the study were invited to complete an age-adapted ages and stages questionnaire (ASQ). Prenatal variables (FETO, disease severity (observed/expected Lung-Head-Ratio; O/E LHR), gestational age at delivery, gender) were retrieved from the medical record, and demographics via the parents (education, profession).

Results:

Of the 87 parents invited, 40 agreed to participate (46%), of whom 20 had FETO. Average age of children was 3.4 ±1.4 years. Overall, 38% children were reported to score below the cut-off in at least one domain, most frequently involving gross motor development (30%). There was no correlation between disease severity (O/E LHR) and abnormal neurologic ASQ-scores. Children who had FETO were born earlier (36.5±2.4 vs 38.0±1.2w; p=0.04) and had more severe pulmonary hypoplasia (o/eLHR: 30.2±5.1 vs 45.8±22.2; p=0.001). There was no difference in total ASQ-scores between children treated with FETO and those managed expectantly during pregnancy (39 vs 40%; p=0.9), neither in its subcategories (communication, gross and fine motor-function, problem solving, social interaction).

Conclusion:

In this cohort, children with prenatally diagnosed CDH were at increased for neurologic developmental delay. We did not find a correlation with disease severity. Children who underwent FETO prenatally, hence who had more severe hypoplasia and were born earlier, had comparable outcomes to those who did not.