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Morphogenesis of canine Chiari malformation and secondary syringomyelia: Disorders of cerebrospinal fluid circulation

Knowler, Susan P, Galea, Gabriel L and Rusbridge, Clare (2018) Morphogenesis of canine Chiari malformation and secondary syringomyelia: Disorders of cerebrospinal fluid circulation Frontiers in Veterinary Science, 5, 171.

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Abstract

Chiari-like Malformation (CM) and secondary syringomyelia (SM), as well as their analogous human conditions, is a complex developmental condition associated with pain and accompanying welfare concerns. CM/SM is diagnosed ever more frequently, thanks in part to the increased availability of magnetic resonance imaging in veterinary medicine. Research over the last two decades has focused primarily on its pathophysiology relating to overcrowding of the cranial caudal fossa. More recent characterizations of CM/SM include brachycephaly with osseous reduction and neural parenchymal displacement involving the entire brain and craniocervical junction to include rostral flattening, olfactory bulb rotation, increased height of the cranium, reduced cranial base with spheno-occipital synchondrosis angulation, reduced supraoccipital and interparietal crest and rostral displacement of the axis and atlas with increased odontoid angulation. The most shared manifestation of CM is the development of fluid-filled pockets (syrinx, syringes) in the spinal cord that can be readily quantified. Dogs with symptomatic CM without SM have a reduced basioccipital bone, compensatory increased cranial fossa height with displaced parenchyma whereby the cerebellum is invaginated beneath the occipital lobes but without compromising cerebrospinal fluid channels enough to cause SM. Thus, broadly defined, CM might be described as any distortion of the skull and craniocervical junction which compromises the neural parenchyma and cerebrospinal fluid circulation causing pain and/or SM. The etiology of CM is multifactorial, potentially including genetically-influenced, breed-specific abnormalities in both skeletal and neural components. Since causation between specific morphologic changes and SM or clinical signs is unproven, CM might be more appropriately considered as a brachycephalic obstructive CSF channel syndrome (BOCCS) rather than a single malformation. Understanding the normal development of the brain, skull and craniocervical junction is fundamental to identifying deviations which predispose to CM/SM. Here we review its anatomical, embryological, bio-mechanical, and genetic underpinnings to update the profession’s understanding of this condition and meaningfully inform future research to diminish its welfare impact.

Item Type: Article
Divisions : Faculty of Health and Medical Sciences > School of Veterinary Medicine
Authors :
NameEmailORCID
Knowler, Susan P
Galea, Gabriel L
Rusbridge, Clarec.rusbridge@surrey.ac.uk
Date : 27 July 2018
Funders : Wellcome Trust
DOI : 10.3389/fvets.2018.00171
Copyright Disclaimer : Copyright © 2018 Knowler, Galea and Rusbridge. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
Uncontrolled Keywords : Arnold Chiari malformation, occipital hypoplasia, craniocervical junction, craniovertebral junction (CVJ), cerebrospinal fluid (CSF), brachycephaly, caudal occipital malformation syndrome (COMS)
Depositing User : Melanie Hughes
Date Deposited : 14 Aug 2018 07:55
Last Modified : 11 Dec 2018 11:24
URI: http://epubs.surrey.ac.uk/id/eprint/848916

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