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.

中文翻译：

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脑异常对脊柱裂患者认知结果的影响：系统评价

背景脊柱裂是影响中枢神经系统的最常见的先天性出生缺陷。鉴于大脑异常的频繁关联，脊柱裂不是中枢神经系统的单一发育异常。脊柱裂患者在认知任务上的表现通常低于平均水平。据推测，相关的大脑异常也会对脊柱裂患者的认知产生负面影响。目的 本研究旨在回顾脑异常对脊柱裂患者认知结果的影响。方法 对多个数据库进行系统搜索，包括 Pubmed、Embase、Web of Science 和 Cochrane Central Register of Controlled Trials。所有相关的主要研究文章都包括在内。所有纳入的文章都使用批判性评估清单进行方法学评估。结果 本系统评价共纳入 27 篇文章。发现了不同的大脑异常对认知的显着影响。更具体地说，发现脑积水、Chiari II 型畸形和胼胝体异常、中央执行网络、默认模式网络、皮质厚度和回旋、穹窿、灰质体积和总脑体积对认知结果有显着影响。脑脊液分流的存在也与认知呈负相关。Chiari 畸形 II 型减压和脑脊液分流并发症的结果不一致。结论 相关的脑异常对脊柱裂患者的认知结果有显着影响。