Department of Neurosurgery, School of Medicine, University of Antioquia, Medellin, Colombia.
Division of Neurosurgery, Department of Medicine, Federal University of São Carlos, UFSCAR, Sao Carlos, Brazil.
Childs Nerv Syst. 2022 Sep;38(9):1683-1688. doi: 10.1007/s00381-022-05551-2. Epub 2022 May 19.
Occipital encephalocele is a brain malformation that has been remotely associated with Dandy-Walker; only case reports and very small series have been published so far; therefore, their behavior and management are still under investigation. The goal of the present case-based review is to provide a summary of the state of the art in this association.
The pertinent literature has been reviewed, and an exemplary case has been reported (an 11-month-old female with Dandy-Walker malformation and occipital encephalocele). So far, 33 cases have been described, with a mean age at surgery of 5, 1 day). The majority of the cases tend to present with hydrocephalus. There are no specific surgery approaches or global consensus about this association. The management possibly relies on surgery with shunt or encephalocele excision but without a dedicated protocol yet.
The clinical research on occipital encephalocele in association with Dandy-Walker malformation is just at the beginning. New targets and wide-ranging clinical trials are needed to get an optimal management protocol.
枕部脑膨出是一种脑部畸形，与 Dandy-Walker 畸形有一定关联，但目前仅报道过一些病例报告和极小型系列病例，因此，其行为和处理方式仍在研究中。本基于病例的综述旨在对该关联的最新技术进行总结。
我们查阅了相关文献，并报告了一个典型病例（一名 11 个月大的女性，患有 Dandy-Walker 畸形和枕部脑膨出）。迄今为止，已描述了 33 例病例，手术时的平均年龄为 5.1 天）。大多数病例往往表现为脑积水。目前针对这种关联，尚无特定的手术方法或全球共识。该疾病的治疗可能依赖于分流或脑膨出切除术，但尚未制定专门的方案。
关于 Dandy-Walker 畸形伴发枕部脑膨出的临床研究才刚刚开始。需要新的目标和广泛的临床试验来制定最佳的管理方案。
