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Neurodevelopmental outcomes at 5years of age in congenital diaphragmatic hernia
Enrico Danzer1, Casey Hoffman1, Jo Ann D'Agostino1
1The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Insights
Congenital diaphragmatic hernia (CDH) survivors show average neurodevelopment at 5 years, but higher rates of borderline/low IQ and behavioral issues like autism. Disease severity predicts long-term impairments.
Area of Science:
- Pediatric Surgery
- Neurodevelopmental Pediatrics
- Congenital Anomalies
Background:
- Congenital diaphragmatic hernia (CDH) is a complex birth defect requiring significant medical intervention.
- Long-term neurodevelopmental outcomes in CDH survivors are crucial for assessing quality of life and guiding care.
- Previous studies highlight potential developmental challenges, necessitating detailed evaluation at school age.
Purpose of the Study:
- To comprehensively evaluate neurodevelopmental sequelae in children with CDH at 5 years of age.
- To identify specific cognitive, academic, and behavioral domains affected in CDH survivors.
- To determine the prevalence of conditions like autism spectrum disorder in this population.
Main Methods:
- A cohort of 35 CDH patients was assessed at a median age of 5 years.
- Neurodevelopmental outcomes included cognition (WPPSI), visual-motor integration, academic achievement (Woodcock-Johnson), and behavior (CBCL).
- Scores were compared to normative data, with statistical analysis to identify risk factors for poorer outcomes.
Main Results:
- While mean IQ scores were within the average range, significantly higher rates of borderline and extremely low IQ scores were observed in CDH children compared to norms.
- Visual-motor integration was below average, and increased rates of emotionally reactive and pervasive developmental problems were noted.
- Autism spectrum disorder was diagnosed in 11% of CDH survivors, a significantly higher prevalence than in the general population. Prolonged NICU stay, intubation, and pulmonary hypertension were associated with worse cognitive outcomes.
Conclusions:
- The majority of CDH children achieve average neurodevelopmental outcomes by age 5.
- However, CDH survivors face significantly increased risks for borderline/low IQ, specific behavioral problems, and autism.
- Disease severity and early neurological dysfunction are key predictors of long-term neurodevelopmental impairments in CDH patients.
Objective:
To evaluate neurodevelopmental sequelae in congenital diaphragmatic hernia (CDH) children at 5years of age.
Materials And Methods:
The study cohort of 35 CDH patients was enrolled in our follow-up program between 06/2004 and 09/2014. The neurodevelopmental outcomes assessed at a median of 5years (range, 4-6) included cognition (Wechsler Preschool and Primary Scale of Intelligence [WPPSI], n=35), Visual-Motor-Integration (n=35), academic achievement (Woodcock-Johnson Tests of Achievement, n=25), and behavior problems (Child Behavior Check List [CBCL], n=26). Scores were grouped as average, borderline, or extremely low by SD intervals.
Results:
Although mean Full (93.9±19.4), Verbal (93.4±18.4), and Performance (95.2±20.9) IQ were within the expected range, significantly more CDH children had borderline (17%) and extremely low (17%) scores in at least one domain compared to normative cohorts (P<0.02). The Visual-Motor-Integration score was below population average (P<0.001). Academic achievement scores were similar to expected means for those children who were able to complete testing. CBCL scores for the emotionally reactive (23%) and pervasive developmental problems scales (27%) were more likely to be abnormal compared to normal population scores (P=0.02 and P=0.0003, respectively). Autism was diagnosed in 11%, which is significantly higher than the general population (P<0.01). Univariate analysis suggests that prolonged NICU stay, prolonged intubation, tracheostomy placement, pulmonary hypertension, autism, hearing impairment, and developmental delays identified during infancy are associated with worse cognitive outcomes (P<0.05).
Conclusion:
The majority of CDH children have neurodevelopmental outcomes within the average range at 5years of age. However, rates of borderline and extremely low IQ scores are significantly higher than in the general population. CDH survivors are also at increased risk for developing symptoms of emotionally reactive and pervasive developmental problems. Risk of autism is significantly elevated. Disease severity and early neurological dysfunction appear to be predictive of longer-term impairments.
