Abstract
BackgroundChiari-like malformation (CM) and syringomyelia is a neurological disease complex with high prevalence in cavalier King Charles spaniels (CKCS). The natural progression of this disease with time has not been described. The objectives of this study were to i) determine if syringomyelia progresses with time ii) determine if features of craniocrebral morphology previously associated with CM are progressive (including caudal cranial fossa volume, caudal cranial fossa parenchymal volume, ventricular dimensions, height of the foramen magnum and degree of cerebellar herniation). A retrospective morphometric analysis was undertaken in 12 CKCS with CM for which repeat magnetic resonance images were available without surgical intervention.ResultsThe maximal syrinx width, height of the foramen magnum, length of cerebellar herniation and caudal cranial fossa volume increased over time. Ventricular and caudal fossa parenchymal volumes were not significantly different between scans.ConclusionsThe results of this study suggest that syringomyelia progresses with time. Increased caudal cranial fossa volume may be associated with active resorption of the supraoccipital bone, which has previously been found in histology specimens from adult CKCS. We hypothesise that active resorption of the supraoccipital bone occurs due to pressure from the cerebellum. These findings have important implications for our understanding of the pathogenesis and variable natural clinical progression of CM and syringomyelia in CKCS.
Highlights
Chiari-like malformation (CM) and syringomyelia is a neurological disease complex with high prevalence in cavalier King Charles spaniels (CKCS)
Ten of twelve dogs presented for the first scan with at least one clinical sign that could be attributable to CM/ SM (83.3%)
There was no significant difference (p=0.188) in caudal cranial fossa (CCF) parenchymal volume (87.20% ± 3.09 vs 88.59% ± 1.97%) or volume of the ventricluar system (p=0.188) between the first and second scans (3.21%, 2.00 - 14.34 vs 2.51%, 1.602 - 17.20%, Figure 3). These results suggest that SM lesion width increases in CKCS with CM with time
Summary
Chiari-like malformation (CM) and syringomyelia is a neurological disease complex with high prevalence in cavalier King Charles spaniels (CKCS). The objectives of this study were to i) determine if syringomyelia progresses with time ii) determine if features of craniocrebral morphology previously associated with CM are progressive (including caudal cranial fossa volume, caudal cranial fossa parenchymal volume, ventricular dimensions, height of the foramen magnum and degree of cerebellar herniation). In cavalier King Charles spaniels (CKCS) the condition has a complex oligogenic trait of moderately high heritability [2,3,4,5]. Other reported abnormalities include occipital bone hypoplasia/dysplasia or a ‘shallow’ occipital bone [8], kinking of the medulla and malformations of the craniocervical junction [8,9,10], ventriculomegaly or hydrocephalus [11] and syringomyelia (SM) [6]. The pathogenesis of CM/SM is often investigated concurrently
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