Hydrocephalus in Dandy–Walker malformation

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 clas...

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Bibliographic Details
Published in:Child's nervous system 2011-10, Vol.27 (10), p.1665-1681
Main Authors: Spennato, Pietro, Mirone, Giuseppe, Nastro, Anna, Buonocore, Maria Consiglio, Ruggiero, Claudio, Trischitta, Vincenzo, Aliberti, Ferdinando, Cinalli, Giuseppe
Format: Article
Language:English
Subjects:
Cranial Fossa, Posterior - pathology
Cranial Fossa, Posterior - surgery
Dandy-Walker Syndrome - complications
Dandy-Walker Syndrome - diagnosis
Dandy-Walker Syndrome - embryology
Dandy-Walker Syndrome - surgery
Endoscopy - methods
Humans
Hydrocephalus - diagnosis
Hydrocephalus - embryology
Hydrocephalus - etiology
Hydrocephalus - pathology
Magnetic Resonance Imaging
Medicine
Medicine & Public Health
Neurosciences
Neurosurgery
Special Annual Issue
Ventriculostomy - methods
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Summary: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.
ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-011-1544-4