Child's Nervous System

, 27:1665 | Cite as

Hydrocephalus in Dandy–Walker malformation

  • Pietro Spennato
  • Giuseppe Mirone
  • Anna Nastro
  • Maria Consiglio Buonocore
  • Claudio Ruggiero
  • Vincenzo Trischitta
  • Ferdinando Aliberti
  • Giuseppe Cinalli
Special Annual Issue

Abstract

Introduction

Even if the first description of Dandy–Walker dates back 1887, difficulty in the establishment of correct diagnosis, especially concerning differential diagnosis with other types of posterior fossa CSF collection, still persists. Further confusion is added by the inclusion, in some classification, of different malformations with different prognosis and therapeutic strategy under the same label of “Dandy–Walker”.

Methods

An extensive literature review concerning embryologic, etiologic, pathogenetic, clinical and neuroradiological aspects has been performed. Therapeutic options, prognosis and intellectual outcome are also reviewed.

Conclusion

The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a “real” Dandy–Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.

Keywords

Dandy–Walker malformation Hydrocephalus CNS malformation Cerebro-spinal fluid dynamics Intellectual outcome Neuroendoscopy Endoscopic third ventriculostomy 

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Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Pietro Spennato
    • 1
  • Giuseppe Mirone
    • 1
  • Anna Nastro
    • 2
  • Maria Consiglio Buonocore
    • 2
  • Claudio Ruggiero
    • 1
  • Vincenzo Trischitta
    • 1
  • Ferdinando Aliberti
    • 1
  • Giuseppe Cinalli
    • 1
  1. 1.Department of Pediatric NeurosurgerySantobono Children’s HospitalNaplesItaly
  2. 2.Department of NeuroradiologySantobono Children’s HospitalNaplesItaly

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