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Child's Nervous System

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01-07-2018 | Case Report

Dandy-Walker malformation and syringomyelia: a rare association

Authors: Valentina Baro, Renzo Manara, Luca Denaro, Domenico d’Avella

Published in: Child's Nervous System | Issue 7/2018

Abstract

Purpose

Dandy-Walker malformation is a rare condition due to imperforation of the Blake’s pouch during intrauterine brain development, usually leading to early severe hydrocephalus. The association with holocord syringomyelia is rare, and from the Gardner’s first report in 1957, only 23 cases have been described, mostly from autopsy series and pre-MRI period. Besides a worsening of clinical picture, its occurrence generates some concern about the best surgical treatment that varies widely among the literature reports.

Methods

An 11-year-old girl with Dandy-Walker malformation presented with a holocord syrinx due to the herniation of the lower pole of the posterior fossa cyst through the foramen magnum.

Results

After an unsuccessful shunt revision, she underwent a cystoperitoneal shunt with regression of the syrinx and of neurological symptoms at the 12-month follow-up.

Conclusions

Previous literature about pathogenesis, treatment, and follow-up is discussed and summarized.

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Title: Dandy-Walker malformation and syringomyelia: a rare association
Authors: Valentina Baro
Renzo Manara
Luca Denaro
Domenico d’Avella
Publication date: 01-07-2018
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 7/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-018-3773-2

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Dandy-Walker malformation and syringomyelia: a rare association

Abstract

Purpose

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusions

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