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Published in: Child's Nervous System 9/2013

01-09-2013 | Special Annual Issue

Myelomeningocele: the management of the associated hydrocephalus

Authors: G. Tamburrini, P. Frassanito, K. Iakovaki, F. Pignotti, C. Rendeli, D. Murolo, C. Di Rocco

Published in: Child's Nervous System | Issue 9/2013

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Abstract

Background

The pathogenesis of the hydrocephalus associated with myelomeningocele (MMC) has been the subject of an extensive number of studies. The contemporary reduction of the incidence of the Chiari II malformation and of the associated active hydrocephalus after closure of the spinal defect in utero is in line with previous studies suggesting a prominent role of the posterior cranial fossa abnormalities, where even the increased venous pressure might be at least mostly a consequence of the constriction of the posterior cranial fossa structures. Pure absorptive abnormalities however coexist, the main ones documented to be abnormal cisternal spaces and peculiar cerebrospinal fluid chemical features.

Materials and methods

We reviewed the pertinent literature concerning the pathogenesis and management of the hydrocephalus associated to MMC. We also reviewed our personal experience in managing the hydrocephalus in such patients through an endoscopic third ventriculostomy.

Results and conclusions

The literature review demonstrated an overall reduction in more recent series of children with MMC needing to be treated for the associated hydrocephalus postnatally, questioning the role of the prenatal care of the disease in this context. Less severe conditions and a more conservative neurosurgical attitude have certainly contributed to the reduction of the reported active postnatal hydrocephalus rate. Long-term cognitive evaluation of the children with MMC that we managed with an endoscopic third ventriculocisternostomy (ETV) as primary as well as secondary procedure did not demonstrate significant differences in the outcome compared with non-complicated extrathecally shunted children, favouring ETV as a valuable option in this subset of patients.
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Metadata
Title
Myelomeningocele: the management of the associated hydrocephalus
Authors
G. Tamburrini
P. Frassanito
K. Iakovaki
F. Pignotti
C. Rendeli
D. Murolo
C. Di Rocco
Publication date
01-09-2013
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 9/2013
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-013-2179-4

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