Long‐term neuroimaging and neurological outcome of fetal spina bifida aperta after postnatal surgical repair

Abstract

Objective Parents faced with the choice between postnatal management and prenatal surgery for spina bifida need to have up‐to‐date information on the expected outcomes. The aim of this study was to report the long‐term physical and neurological outcomes of infants with prenatally diagnosed isolated spina bifida that underwent postnatal surgical repair and were managed by a multidisciplinary team from a large tertiary center. Methods This was a retrospective cohort study of all cases of fetal spina bifida managed in a tertiary unit between October 1999 and January 2018. All cases of fetal spina bifida from the local health region were routinely referred to the tertiary unit for further perinatal management. Details on surgical procedures and neonatal neurological outcomes were obtained from institutional case records. Ambulatory status, bladder and bowel continence and neurodevelopment were assessed at a minimum of 3 years. Results During the study period, 241 pregnancies with isolated spina bifida were seen in the unit. Of these, 84 (34.9%) women opted to continue with the pregnancy after multidisciplinary counseling by clinicians. Sixty‐seven infants underwent postnatal repair of spina bifida aperta and were included in the analysis. After birth, hindbrain herniation was observed in 91.5% of infants with only seven requiring surgical decompression. Ventriculoperitoneal shunt placement was needed in 64.2% of infants, while normal cognitive development or mild impairment was demonstrated in 85.4% of cases with data for this outcome available, at a mean age of 8 years. Cumulatively, 40% of infants were walking independently or using minor support, and normal or mild impairment of bladder and bowel function was reported in 45.5% and 44.4% of infants, respectively. Conclusions Neurodevelopmental and neurological outcomes between prenatal and postnatal repair are similar. As with fetal surgery, conventional postnatal surgery is associated with the reversal of hindbrain herniation. Similarly, postnatal ventriculoperitoneal shunt placement appears to be required mainly in fetuses without evidence of significant fetal ventriculomegaly. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Management of prenatally diagnosed spina bifida: how do we move ahead?

“…Also in this issue of the Journal are two papers that highlight the pitfalls associated with trying to extrapolate clinically meaningful information from retrospective data. Sileo et al .…”

“…Sileo et al . present results from a retrospective cohort of newborns who were diagnosed with an open neural tube defect in utero and who underwent postnatal surgical repair. Masini et al .…”

“…Sileo et al . report that, of the 241 women with pregnancy affected by an isolated open neural tube defect, only 84 (39%) opted to continue the pregnancy, and, of these, 14 (17%) were excluded, leaving a cohort of 70 pregnancies.…”

“…'s concluding statement that: ‘Future studies focused on neurocognitive follow‐up of spina‐bifida children who undergo surgery compared with those who do not are urgently needed’. The papers by Sileo et al . and Masini et al .…”

“…Also in this issue of the Journal are two papers that highlight the pitfalls associated with trying to extrapolate clinically meaningful information from retrospective data 10 11 . While both groups should be congratulated for assembling reasonably sized cohorts with long-term follow-up, the papers suffer from the major problems inherent to retrospective reviews, i.e.…”