Long-Term Intellectual and Fine Motor Outcomes in Spina Bifida Are Related to Myelomeningocele Repair and Shunt Intervention History

Abstract

Objective:Lifespan outcomes of simultaneous versus sequential myelomeningocele repair and shunt placement or effects of repeated shunt revisions on specific domains of IQ or fine motor dexterity are largely unknown. The current study addressed these gaps in a large cohort of children and adults with spina bifida myelomeningocele (SBM).Methods:Participants between 7 and 44 years of age with SBM and shunted hydrocephalus were recruited from international clinics at two time points. Each participant completed a standardized neuropsychological evaluation that included estimates of IQ and fine motor dexterity. Simultaneous versus sequential surgical repair and number of shunt revisions were examined in relation to long-term IQ and fine motor scores.Results:Simultaneous myelomeningocele repair and shunting were associated with more frequent shunt revisions, as well as to lower Full Scale and verbal IQ scores, controlling for number of shunt revisions. More shunt revisions across study time points were associated with higher nonverbal IQ (NVIQ) scores. No effects were observed on fine motor dexterity.Conclusions:Findings indicate generally greater influence of surgery type over shunt revision history on outcomes in well-managed hydrocephalus. Findings supported apparent, domain-specific benefits of sequential compared to simultaneous surgery across the lifespan in SBM. Higher NVIQ scores with greater number of additional shunt revisions across surgery type supported positive outcomes with effective surgical management for hydrocephalus.

Spina bifida and other neural tube defects.

“…Among adults with SBM characterized by Chiari II malformation and associated hydrocephalus, cognitive outcomes were variable, with attention being the most severely affected domain. Overall cognitive and functional outcomes were most closely related to the severity of congenital brain abnormalities as opposed to shunt malfunction and revisions (Jenkinson et al, 2011;Ware et al, 2020). Evidence of lasting impairments in executive functioning, attention, and visuospatial abilities are also present in those with the less severe Chiari I malformation (which is not associated with SBM), with early language deficits resolving with age (Rogers et al, 2018;Steinberg et al, 2020).…”