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Published on: February 26, 2019
Early childhood neurodevelopmental outcome after open prenatal spina bifida aperta repair
Zehra S Hepp1, Verena M Haas2, Beatrice Latal1,3
1Division of Child Developmental Medicine, University Children's Hospital Zurich, Zurich, Switzerland.
Insights
Children undergoing prenatal spina bifida aperta (SBA) repair show normal cognitive development, though gross motor skills lag. Fine motor skills are age-appropriate, with about half of children walking by age two.
Area of Science:
- Pediatric Neurology
- Developmental Pediatrics
- Fetal Surgery
Background:
- Spina bifida aperta (SBA) is a complex congenital condition requiring early intervention.
- Prenatal surgical repair of SBA aims to improve neurodevelopmental outcomes.
- Assessing long-term neurodevelopmental trajectories is crucial for guiding patient care.
Purpose of the Study:
- To evaluate the neurodevelopmental outcomes in children who underwent open prenatal repair for spina bifida aperta (SBA).
- To identify potential risk factors influencing developmental trajectories post-prenatal SBA repair.
- To correlate findings from different developmental assessment scales.
Main Methods:
- A cohort of 130 fetuses underwent open prenatal SBA repair between 2010-2019.
- Neurodevelopmental assessments included the Griffiths Mental Developmental Scales at 1 year and Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) at 2 years.
- Descriptive statistics and multiple linear regression analyses were employed to analyze data.
Main Results:
- Bayley-III cognition scores were within normal limits but lower than population norms (mean=95.15, SD=14.683).
- Fine motor development was age-appropriate, while gross motor development was significantly delayed (p<0.001).
- At 2 years, 50.8% of children achieved ambulation.
Conclusions:
- Open prenatal SBA repair is associated with favorable cognitive outcomes in the low-average range.
- Gross motor function remains a significant challenge, whereas fine motor skills are typically developed.
- Neurodevelopmental assessments at 1 and 2 years show significant correlations, aiding outcome prediction.
Aim:
To investigate neurodevelopmental outcome of children with open prenatal spina bifida aperta (SBA) repair.
Method:
Prenatal SBA repair was performed in 130 fetuses at the Zurich Center between 2010 and 2019. Seventy-seven children underwent 1 year assessment with the Griffiths Mental Developmental Scales (Griffiths) and 65 with the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) at 2 years. Anatomical and functional level and ambulation status were assessed. Descriptive statistics and multiple linear regression analyses for risk factors were performed.
Results:
The Bayley-III cognition composite score in children with prenatal SBA repair was within normal limits but lower compared to population norms (mean=95.15, SD=14.683 vs norm=100, SD=15, p=0.01). Fine motor development (mean=9.58, SD=2.744, p=0.227) was typical while gross motor development was lower than the norm (mean=3.02, SD=2.758 vs norm=10, SD=3, p<0.001). Griffiths developmental quotient subscales correlated significantly with corresponding Bayley-III scores (all p<0.001, r=0.519-0.594). At 2 years, 50.8% could walk.
Interpretation:
Children with non-trial open prenatal SBA repair show favourable cognitive outcome in the low-average range at 1 and 2 years of age. While gross motor function remained delayed, fine motor function was age appropriate. The correlation between Griffiths and Bayley-III allows a prediction about neurodevelopmental outcome at the age of 1 year. What this paper adds Children with non-trial open prenatal spina bifida repair show favourable cognitive outcome. Gross motor function remains impaired, while fine motor function is age appropriate. At 2 years of age, 50.8% of children were walking. Neurodevelopmental testing correlated between 1 (Griffiths Mental Developmental Scales) and 2 (Bayley Scales of Infant and Toddler Development, Third Edition) years.

