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Child's Nervous System

, Volume 34, Issue 7, pp 1401–1406 | Cite as

Dandy-Walker malformation and syringomyelia: a rare association

  • Valentina Baro
  • Renzo Manara
  • Luca Denaro
  • Domenico d’Avella
Case Report

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.

Keywords

Dandy-Walker malformation Syringomyelia Syrinx cyst 

Abbreviations

DWM

Dandy-Walker malformation

MRI

Magnetic resonance imaging

CT

Computed tomography

VPS

Ventriculoperitoneal shunt

CPS

Cystoperitoneal shunt

CSF

Cerebospinal fluid

IVH

Intraventricular hemorrhage

C1M-LM

Chiari 1 malformation-like mechanism

Notes

Compliance with ethical standards

Conflict of interest

The authors report no conflict of interest concerning the materials or methods used in this study or the findings disclosed in this paper.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Academic Neurosurgery, Department of NeurosciencesUniversity of Padova Medical SchoolPadovaItaly
  2. 2.Department of Medicine, Surgery and Dentistry, Scuola Medica SalernitanaUniversity of SalernoSalernoItaly

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