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Occipital encephalocele associated with Dandy-Walker malformation: a case-based review

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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.

Methods and results

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.

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Authors and Affiliations



All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by FG and MB. The first draft of the manuscript was written by FG and DH. Writing — review and editing — was performed by FG, MB, CG, AC, and LM. All authors approved the version to be published. All authors agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors read and approved the final manuscript.

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Correspondence to Felipe Gutierrez.

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The authors declare no competing interests.

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Ethical approval was waived by the local Ethics Committee in view of the retrospective nature of the study, and all the procedures being performed were part of the routine care.

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The mother of the patient was informed in detail prior to the authorization to take the clinical history, with the presence of institutional informed consent duly written and signed by her.

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The authors declare no competing interests.

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Gutierrez, F., Ballestero, M., Herrera, D. et al. Occipital encephalocele associated with Dandy-Walker malformation: a case-based review. Childs Nerv Syst 38, 1683–1688 (2022).

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