Hydrocephalus and Dandy- Walker Malformation: a review

Patricia Alessandra Dastoli Dastoli,Marcia Cristina Da Silva,Marcos Devanir Silva Da Costa,Jardel Mendonça Nicácio,Sergio Cavalheiro,Italo Capraro Suriano

doi:10.46900/apn.v2i3(september-december).44

Patricia Alessandra Dastoli Dastoli, Marcia Cristina Da Silva + Show 4 more

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https://doi.org/10.46900/apn.v2i3(september-december).44

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Abstract

Introduction: Dandy Walker malformation (DWM) is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle leading to an increase of the posterior fossa and superior dislocation of the lateral sinus, tentorium and torcula. Although it is the commonest posterior fossa malformation, its pathogenesis is still not fully understood, making the differential diagnosis with other posterior fossa malformations difficult and as a result the choice of therapeutic strategy. Material and methods: An extensive review of the literature relating to Dandy Walker malformation was performed. Historical, genetic, embryologic, epidemiologic, clinical and radiological presentation, treatment and prognosis were revised. Conclusion: The correct diagnosis of Dandy Walker malformation can be made through careful interpretation of magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) flow studies. The choice of hydrocephalus treatment depends on whether there is aqueduct stenosis. And, although ventriculoperitoneal (VP) shunts have been the treatment of choice for many years, neuroendoscopic techniques such as endoscopic thirdventriculostomy and stent placement are being frequently performed lately.

Highlights

Dandy Walker malformation (DWM) is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle leading to an increase of the posterior fossa and superior dislocation of the lateral sinus, tentorium and torcula
Dandy and Blackfan in 1914 reported the case of a 13month-old girl with severe hydrocephalus associated with a dilated fourth ventricle, hypoplasia of the cerebellar vermis and absence of the foramina of Luschka and Magendie (Fig. 1)
Taggart and Walker reported in 1942 the case of three children presenting with hydrocephalus associated to atresia of the foramina of Luschka and Magendie[4]

Summary

Introduction

Dandy Walker malformation (DWM) is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle leading to an increase of the posterior fossa and superior dislocation of the lateral sinus, tentorium and torcula. It is the commonest posterior fossa malformation, its pathogenesis is still not fully understood, making the differential diagnosis with other posterior fossa malformations difficult and as a result the choice of therapeutic strategy. The goal of this review of the literature is to promote up-to-date understanding of DWM, its definition, the cerebrospinal (CSF) circulation changes that result in hydrocephalus and differential diagnosis in imaging studies. In light of these concepts, the different strategies for the treatment of hydrocephalus employed in the last decades are discussed

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