Cerebral Cortical Changes in Craniosynostosis: A Systematic Review.

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This study aims to review the existing literature on cerebral cortical changes in craniosynostosis during the months of August and September 2023. It focuses on alterations occurring in cases of both syndromic and non-syndromic forms of the disease. In particular, variations in volume, size, and structure (e.g., connectivity) of the cortex are studied. For the present review, a systematic search of the PubMed database was performed using the terms "cerebral cortex"[tw] OR "Cerebral Cortex"[Mesh] AND "Craniosynostoses"[Mesh]. The initial search retrieved 50 articles, which were studied in their entirety. After applying the selection and exclusion criteria, 34 articles were excluded, and finally, 16 qualified and were included in this study. The Anatomical Quality Assessment (AQUA) tool was preferred for the quality assessment of the included publications. Part of the articles used refers to the syndromic form of the disease and discusses temporal lobe and frontal cortex abnormalities, thinning and disproportionate increase in cerebral cortical surface area, and simplified gyroscopic pattern. The remaining articles, referring to non-syndromic craniosynostosis, are focused on neuronal connectivity, grey matter volume, and Sylvian fissure volume. In the existing literature, two theories have been proposed to describe the relationship between craniosynostosis and cortical changes. The deformation theory states that skull deformities result in brain architecture malformations, and the deformity theory supports that brain abnormalities pre-exist and lead to premature fusion of the cranial sutures. The existing data are not sufficient to resolve the above dilemma. Regarding the therapeutic approach, it differs depending on the type of craniosynostosis. Surgery remains the most common method, while innovative treatments are also emerging, including the application of regenerative medicine.