The impact of cerebral anomalies on cognitive outcome in patients with spina bifida: A systematic review

Eur J Paediatr Neurol. 2020 Sep;28:16-28. doi: 10.1016/j.ejpn.2020.07.005. Epub 2020 Jul 28.


Background: Spina bifida is the most common congenital birth defect affecting the central nervous system. Given the frequent association of cerebral anomalies, spina bifida is not a single developmental abnormality of the central nervous system. Patients with spina bifida typically perform below average on cognitive tasks. It has been hypothesized that associated cerebral anomalies as well negatively affect cognition in spina bifida patients.

Objective: This study aims to review the impact of cerebral anomalies on cognitive outcome in patients with spina bifida.

Methods: A systematic search of multiple databases, including Pubmed, Embase, Web of Science and The Cochrane Central Register of Controlled Trials, was performed. All relevant primary research articles were included. All included articles were methodologically evaluated using a critical appraisal checklist.

Results: In total 27 articles were included in this systematic review. A significant impact of different cerebral anomalies on cognition was found. More specifically, hydrocephalus, Chiari malformation type II and anomalies of the corpus callosum, central executive network, default mode network, cortical thickness and gyrification, fornix, grey matter volume and total brain volume were found to have a significant impact on cognitive outcome. The presence of a CSF shunt was also negatively associated with cognition. The results on Chiari malformation type II decompression and CSF shunt complications are inconsistent.

Conclusion: Associated cerebral anomalies have a significant impact on cognitive outcome in patients with spina bifida. The interrelatedness of the different cerebral anomalies makes it difficult to distinguish their individual impact on cognition.

Keywords: Cerebral anomalies; Chiari malformation type II; Cognition; Corpus callosum; Hydrocephalus; Spina bifida.

Publication types

  • Systematic Review

MeSH terms

  • Cognitive Dysfunction / epidemiology*
  • Cognitive Dysfunction / etiology*
  • Female
  • Humans
  • Male
  • Nervous System Malformations / complications*
  • Spinal Dysraphism / complications*