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Child's Nervous System
Published in: Child's Nervous System 11/2015

01-11-2015 | Review Paper

Chiari III malformation: a comprehensive review of this enigmatic anomaly

Authors: Galyna Ivashchuk, Marios Loukas, Jeffrey P. Blount, R. Shane Tubbs, W. Jerry Oakes

Published in: Child's Nervous System | Issue 11/2015

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Abstract

Introduction

Chiari III is one of the rarest of the Chiari malformations and is characterized by a high cervical or low occipital encephalocele and osseous defect with or without spinal cord involvement and may include many of the anatomical characteristics seen in the Chiari II malformation. Herein, we provide a comprehensive review of this rare anomaly as well as a translation of Chiari’s original description.

Methods

We review all reported cases of Chiari III malformation found in the extant literature.

Conclusions

Out of 57 reported cases of Chiari III malformation, encephaloceles were in a high cervical/low occipital position in 23, 8 were in a high cervical position, 17 were in low occipital position, and the position in 9 cases was not reported. The pathogenesis of Chiari III malformation remains unclear. The majority of patients have concomitant hydrocephalus. Brain parts occurring in the sac from the most to least common include the following: cerebellum, occipital lobe, and parietal lobe. The severity of symptoms is correlated with the amount of brain structures within the encephalocele. Neurologic functional outcomes have been varied and depend on the neurological status of the patient before surgery.
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Metadata
Title
Chiari III malformation: a comprehensive review of this enigmatic anomaly
Authors
Galyna Ivashchuk
Marios Loukas
Jeffrey P. Blount
R. Shane Tubbs
W. Jerry Oakes
Publication date
01-11-2015
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 11/2015
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-015-2853-9

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