Early childhood neurodevelopmental outcome after open prenatal spina bifida aperta repair

Abstract

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. 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.

Impact on postoperative, neonatal and 2‐year neurodevelopmental outcomes of UA‐AREDF during and after fetal spina bifida repair

“…We have already published data on early neurodevelopment of our cohort following fetal OSB repair, which showed a favorable outcome in the low-average range at 1 and 2 years of age 34 . Age-appropriate fine motor function was observed, with an expected delay in gross motor function 34 . In this study, we showed that UA-AREDF did not negatively influence the 2-year neurodevelopmental outcome of the children.…”

Expert ultrasonographic evaluation in fetuses with spina bifida: An essential prerequisite to select candidate to fetal surgery

“…In contrast, motor skills were improved in children with open prenatal change in spina bifida (SBA) repair. 7,8 As a consequence, a selection of the fetuses that may benefit of intrauterine surgery is necessary. In this attempt Hérbene José et al 1 despite failed to evidence significant differences in the TVOL between normal and spina bifida fetuses evidenced that in fetuses with spina bifida with presence of lower limb movement higher TVOL values were found compared to fetuses without lower limb movements.…”

“…Despite benefits and risks are still uncertain, long‐term data from the Management of Myelomeningocele Study cohort, published in showed no improvement in cognitive functioning in the prenatal repair group at primary school age compared to the postnatal group. In contrast, motor skills were improved in children with open prenatal change in spina bifida (SBA) repair 7,8 . As a consequence, a selection of the fetuses that may benefit of intrauterine surgery is necessary.…”

Fetal surgery for spina bifida: a great success story in surgical innovation

“…As such, they have scientifically validated, to an acceptable degree, the generalizability of the MOMS findings. Both these retrospective cohort studies suggest that single‐site fetal centers functioning outside of a randomized trial environment, respectively in Europe 3 and in the USA where results from independent centers were aggregated in the National Spina Bifida Patient Registry, 4 can achieve comparable short‐ and medium‐term outcomes to the MOMS trial.…”

“…and Worley et al. have bridged this gap in knowledge by demonstrating different aspects of the effectiveness of fetal SBA surgery 3,4 . As such, they have scientifically validated, to an acceptable degree, the generalizability of the MOMS findings.…”