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Published in: Child's Nervous System 7/2017

01-07-2017 | Review Paper

Hydrocephalus and mucopolysaccharidoses: what do we know and what do we not know?

Authors: Amauri Dalla Corte, Carolina F. M. de Souza, Maurício Anés, Roberto Giugliani

Published in: Child's Nervous System | Issue 7/2017

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Abstract

Introduction

The precise incidence of hydrocephalus in patients with mucopolysaccharidoses (MPS) is hard to determine, because the condition lacks a formal, consensus-based definition. The diagnosis of hydrocephalus depends on symptom profile, presence of neuroimaging features, and the outcome of diagnostic tests. Although numerous techniques are used to identify MPS patients who are most likely to have hydrocephalus and respond to treatment, no definitive method exists to prove diagnosis.

Purpose

The authors propose an algorithm to aid in the diagnosis and management of hydrocephalus in MPS patients.

Conclusions

The theory of venous hypertension associated with the morphological changes in the skull base and craniocervical junction indicate the need for future neuroimaging studies including cerebrospinal fluid (CSF) and venous flow measurements to monitor hydrocephalus progression and select therapeutic interventions in MPS patients. Preoperative planning should also be based on the increased risk of intraoperative and postoperative hemorrhagic complications.
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Metadata
Title
Hydrocephalus and mucopolysaccharidoses: what do we know and what do we not know?
Authors
Amauri Dalla Corte
Carolina F. M. de Souza
Maurício Anés
Roberto Giugliani
Publication date
01-07-2017
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 7/2017
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-017-3476-0

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