Use of Morphometric Mapping to Characterise Symptomatic Chiari-Like Malformation, Secondary Syringomyelia and Associated Brachycephaly in the Cavalier King Charles Spaniel

PLoS One. 2017 Jan 25;12(1):e0170315. doi: 10.1371/journal.pone.0170315. eCollection 2017.

Abstract

Objectives: To characterise the symptomatic phenotype of Chiari-like malformation (CM), secondary syringomyelia (SM) and brachycephaly in the Cavalier King Charles Spaniel using morphometric measurements on mid-sagittal Magnetic Resonance images (MRI) of the brain and craniocervical junction.

Methods: This retrospective study, based on a previous quantitative analysis in the Griffon Bruxellois (GB), used 24 measurements taken on 130 T1-weighted MRI of hindbrain and cervical region. Associated brachycephaly was estimated using 26 measurements, including rostral forebrain flattening and olfactory lobe rotation, on 72 T2-weighted MRI of the whole brain. Both study cohorts were divided into three groups; Control, CM pain and SM and their morphometries compared with each other.

Results: Fourteen significant traits were identified in the hindbrain study and nine traits in the whole brain study, six of which were similar to the GB and suggest a common aetiology. The Control cohort had the most elliptical brain (p = 0.010), least olfactory bulb rotation (p = 0.003) and a protective angle (p = 0.004) compared to the other groups. The CM pain cohort had the greatest rostral forebrain flattening (p = 0.007), shortest basioccipital (p = 0.019), but a greater distance between the atlas and basioccipital (p = 0.002) which was protective for SM. The SM cohort had two conformation anomalies depending on the severity of craniocervical junction incongruities; i) the proximity of the dens (p <0.001) ii) increased airorhynchy with a smaller, more ventrally rotated olfactory bulb (p <0.001). Both generated 'concertina' flexures of the brain and craniocervical junction.

Conclusion: Morphometric mapping provides a diagnostic tool for quantifying symptomatic CM, secondary SM and their relationship with brachycephaly. It is hypothesized that CM pain is associated with increased brachycephaly and SM can result from different combinations of abnormalities of the forebrain, caudal fossa and craniocervical junction which compromise the neural parenchyma and impede cerebrospinal fluid flow.

MeSH terms

  • Animals
  • Arnold-Chiari Malformation / complications
  • Arnold-Chiari Malformation / diagnostic imaging
  • Arnold-Chiari Malformation / genetics
  • Arnold-Chiari Malformation / veterinary*
  • Brain / diagnostic imaging*
  • Brain / pathology
  • Breeding
  • Cephalometry*
  • Cervical Vertebrae / diagnostic imaging*
  • Cervical Vertebrae / pathology
  • Craniosynostoses / diagnostic imaging
  • Craniosynostoses / genetics
  • Craniosynostoses / veterinary*
  • Discriminant Analysis
  • Dog Diseases / diagnostic imaging*
  • Dog Diseases / genetics
  • Dog Diseases / pathology
  • Dogs
  • Female
  • Foramen Magnum / diagnostic imaging
  • Foramen Magnum / pathology
  • Genetic Predisposition to Disease
  • Magnetic Resonance Imaging*
  • Male
  • Observer Variation
  • Olfactory Bulb / diagnostic imaging
  • Olfactory Bulb / pathology
  • Phenotype
  • Reproducibility of Results
  • Retrospective Studies
  • Rhombencephalon / diagnostic imaging*
  • Rhombencephalon / pathology
  • Skull / diagnostic imaging*
  • Skull / pathology
  • Skull Base / diagnostic imaging
  • Skull Base / pathology
  • Syringomyelia / diagnostic imaging
  • Syringomyelia / etiology
  • Syringomyelia / genetics
  • Syringomyelia / veterinary*

Grants and funding

Cavalier Matters Charity (http://www.cavaliermatters.org/) Reg No 1141674 funded SPK’s doctoral research. CC was supported by the BSAVA Petsavers Millennium Award, Woodrow House, Quedgeley GL22AB http://www.petsavers.org.uk/ and, together with The Dogs Trust Charity https://www.dogstrust.org.uk/ Reg. No. 227523 & SC037843, contributed towards the cost of leasing the DICOM viewing software package MIMICS 14.12 Materialise (Technologielaan 15 3001 Leuven Belgium). Henny van/der Berg donated the photo morphing software Abrosoft FantaMorph. Rupert’s Fund (www.rupertsfund.com) and For the Love of Ollie (www.fortheloveofollie.com) provided financial support for health screening. AKM is an independent statistical consultant operating professionally as a sole trader under the company name of akm-stats and did not receive any salary or other remittance for his contribution in this investigation. Fitzpatrick Referrals Ltd, provided support in the form of salaries and materials for authors CD, JJ, AT and CR and the Goddard Veterinary Group provided the salary for SG. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.