Ventriculomegaly in Cavalier King Charles Spaniels with Chiari-like malformation: relationship with clinical and imaging findings

Tirrito, Federica; Cozzi, Francesca; Bonaldi, Martina; Corazzo, Stefania; Contiero, Barbara; Lombardo, Rocco

doi:10.1292/jvms.22-0134

Cited by 8 publications

(9 citation statements)

References 35 publications

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“…This could depend on the increasing number of CM1 diagnoses and on the more extensive diagnostic work-up that epileptic (and CM1) patients currently undergo. Such an epidemiological association is also well-known in Cavalier King Charles Spaniels dogs (CM1 77%, epilepsy 28%) where, however, no relevant correlations between the conditions have been found [ 18 ]. Similar considerations can be made about the prevalence of CM1 in epileptic subjects, which was as high as 12% in the present series but about 5% in historical series [ 19 ].…”

Section: Discussionmentioning

confidence: 87%

Chiari 1 Malformation and Epilepsy in Children: A Missing Relationship

Massimi

Palombi

Contaldo

et al. 2022

JCM

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Purpose: Once believed a result of pathophysiological correlations, the association between Chiari 1 malformation (CM1) and epilepsy has since been considered as a coincidence, due to missing etiologic or clinical matching points. At present, the problem is being newly debated because of the increasing number of CM1 diagnoses, often among children with seizures. No specific studies on this topic are available yet. The present study aimed at updating the information on this topic by reporting on a series of children specifically enrolled and retrospectively analyzed for this purpose. Methods: All children admitted between January 2015 and June 2020 for epilepsy and CM1 were considered (Group 1). They were compared with children admitted in the same period for symptoms/signs related to CM1 and/or syringomyelia (Group 2). Syndromic patients were excluded, as well as those with tumoral or other overt intracranial lesions. All patients received a complete preoperative work-up, including MRI and EEG. Symptomatic children with CM1/syringomyelia were operated on. The pertinent literature was reviewed. Results: Group 1 was composed of 29 children (mean age: 6.2 years) showing CM1 and epilepsy with several types of seizures. A share of 27% had CM1-related symptoms and syringomyelia. The mean tonsillar ectopia was 7.5 mm. Surgery was performed in 31% of cases. Overall, 62% of children are currently seizure-free (including 5/9 children who were operated on). Tonsillar herniation and syringomyelia regressed in 4/9 cases and 4/8 cases, improved in 4/9 cases and 3/8 cases, and remained stable in 1/9 and 1/8 cases, respectively. CM1 signs/symptoms regressed completely in 6/8 cases and improved or remained stable in one case in each of the two remaining patients. Group 2 consisted of 77 children (mean age: 8.9 years) showing symptoms of CM1 (75%) and/or syringomyelia (39%). The mean tonsillar ectopia was 11.8 mm. Non-specific EEG anomalies were detected in 13 children (17%). Surgery was performed in 76.5% of cases (18 children were not operated on because of oligosymptomatic). Preoperative symptoms regressed in 26%, improved in 50%, remained stable 22%, and worsened in 2%; CM1 radiologically regressed in 39%, improved in 37%, remained unchanged in 22%, and worsened in 2%; and syringomyelia/hydromyelia regressed in 61%, improved in 30%, and was stable in 9%. No statistically significant differences between the two groups were detected regarding the M/F ratio, presence of syringomyelia/hydromyelia, or CM1/syringomyelia outcome; moreover, no correlation occurred between seizure-free condition and PF decompression in Group 1, or between disappearance of EEG anomalies and PF decompression in Group 2. A significant difference between the two groups was noticed regarding the mean age at admission (p = 0.003), amount of tonsillar herniation (p < 0.00001), and PF decompression (p = 0.0001). Conclusions: These findings do not support clinical correlations between CM1 and epilepsy. Their course depends on surgery and antiepileptic drugs, respectively. The analysis of the literature does not provide evidence of a relationship between seizures and cerebellar anomalies such as CM1. Rather than being linked to a syndrome that could explain such an association, the connection between the two now has to be considered to be random.

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Section: Discussionmentioning

confidence: 87%

Chiari 1 Malformation and Epilepsy in Children: A Missing Relationship

Massimi

Palombi

Contaldo

et al. 2022

JCM

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“…Chiari-like malformation (CM also called canine Chiari) and syringomyelia (SM), first reported in 1997 ( 1 ), are most common ( 2 ). Other neurological abnormalities occasionally observed are ventriculomegaly ( 3–5 ), medullary kinking ( 6 ), atlanto-axial problems ( 6 , 7 ), myoclonus ( 8 ), Medium-Chain Acyl-CoA Dehydrogenase Deficiency ( 9 ), and episodic falling ( 10 , 11 ). The last frequently observed abnormality is the so-called glue ear (mucous in the tympanic bullae) and, by some authors, described as primary secretory otitis media (PSOM) ( 12 ).…”

Section: Introductionmentioning

confidence: 99%

The effect of MRI-based screening and selection on the prevalence of syringomyelia in the Dutch and Danish Cavalier King Charles Spaniels

Limpens,

Smits,

Fieten

et al. 2024

Front. Vet. Sci.

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IntroductionSyringomyelia (SM) is a heritable disorder causing a fluid filled cavity (FFC) in the spinal cord with a reported overall prevalence of 39 to 46% in the Cavalier King Charles Spaniels (CKCS). Breeders started screening their CKCS with MRI in the Netherlands since 2004 and in Denmark since 2015. The goal of this study was to evaluate the effect of MRI-based selection in breeding on the prevalence of SM.MethodMRI scans of 2,125 purebred CKCS were available. SM was defined as having a visible FFC in the spinal cord. The prevalence of SM per year of birth was calculated, and a logistic regression was used to evaluate the affected status of offspring from affected versus unaffected parents and age category of the parent and study the combined effect of parental status and age-category to evaluate the effect on the affected status of the offspring.ResultsThe mean FFC in affected CKCS was 2.03 ± 1.47 mm and ranged from 0.5 to 9 mm (median of 1.5 mm). An age effect exists as older CKCS, which has a higher frequency of being affected compared with younger CKCS. There was no significant sex predilection for SM in this dataset. The mean prevalence of SM decreased slightly from 38% (2010–2014; 2.8 ± 1.3 years of age (mean ± sd); median 2.6 years) to 27% (2015–2019; 2.4 ± 1.2 years of age; median 2.1 years) in the screened population of CKCS (p = 4.3e-07). Breeding with two affected parents increased the odds ratio with 3.08 for producing affected offspring (95% CI 1.58–6.04) compared with breeding with unaffected parents.DiscussionMRI-based screening and selection against SM led to a minimal decrease in the prevalence of SM in the Dutch and Danish CKCS population. Breeding with dogs with SM significantly increases the risk of affected offspring. As the disorder is progressive with age, and based on the results of this study, MRI-based screening for all CKCS is recommended at an age of 3 years or older, and to reduce SM more effectively, CKCS affected with SM should not be used for breeding.

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“…Chiari-like malformation (CLM) predominantly affects Cavalier King Charles Spaniels and is known as Chiari Type 1 malformation (CM1) in humans [1][2][3][4]. This condition is characterized by a morphologically malformed skull and craniocervical junction, resulting in a relatively small caudal cranial fossa (CCF) and caudal displacement of the cerebellum into the foramen magnum due to overcrowding of the CCF [1][2][3][4]. Herniation of the cerebellum due to skull insufficiency causes deformation of the cerebellum, prompted by insufficient skull capacity, leading to deformation of the craniocervical junction, including loss of the cisterna magna.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of foramen magnum decompression with and without cranioplasty in a rat model of Chiari-like malformation

Jung,

Cho,

Kim

et al. 2024

Preprint

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In veterinary medicine, canine Chiari-like malformation (CLM) disease is surgically managed through foramen magnum decompression (FMD) with cranioplasty. This study aimed to assess the efficacy of cranioplasty surgery by establishing a rat CLM model and then applying FMD with and without cranioplasty and comparing the outcomes. Twenty-four 8-week-old male Sprague-Dawley rats underwent surgery to induce CLM by reducing the caudal cranial fossa volume, mimicking cerebellum herniation. The rats were randomly and equally assigned to three groups: a control group (induced CLM), an FO group (induced CLM rats undergoing FMD only), and a CR group (induced CLM rats undergoing FMD with cranioplasty). At 11 weeks of age, the FO and CR groups underwent FMD surgery. Four weeks later, magnetic resonance imaging (MRI) was used to measure the cisterna magna volume to assess surgical outcomes. Post-surgery MRI revealed that the mean cisterna magna volume was 23.82 ± 1.70, 34.88 ± 4.39, and 29.48 ± 2.20 mm3 in the control, FO, and CR groups, respectively. There was a significant increase in the cisterna magna volume in the FO and CR groups compared to that in the control group (p < 0.05), with the FO group showing a significantly greater increase than the CR group (p < 0.05). These findings suggest that FMD surgery alone is more effective at restoring the cisterna magna volume than FMD surgery with cranioplasty. FMD surgery alone resulted in a greater increase in cisterna magna volume than FMD with cranioplasty in our rat CLM model, suggesting that FMD alone may be more effective to treat canine CLM. These findings challenge the typical approach of combining FMD with cranioplasty in treating canine CLM disease and underscore the need for further investigation into optimizing surgical techniques for CLM.

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Ventriculomegaly in Cavalier King Charles Spaniels with Chiari-like malformation: relationship with clinical and imaging findings

Cited by 8 publications

References 35 publications

Chiari 1 Malformation and Epilepsy in Children: A Missing Relationship

Chiari 1 Malformation and Epilepsy in Children: A Missing Relationship

The effect of MRI-based screening and selection on the prevalence of syringomyelia in the Dutch and Danish Cavalier King Charles Spaniels

Efficacy of foramen magnum decompression with and without cranioplasty in a rat model of Chiari-like malformation

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