Chiari-like malformation and syringomyelia in normal cavalier King Charles spaniels: a multiple diagnostic imaging approach

Objectives : The objectives of the study were (1) to report the incidence of Chiari‐like malformation and syringomyelia in a normal French cavalier King Charles spaniel breeding population; (2) to precise the standard computed tomography dimensions of the caudal fossa and (3) to investigate the use of ultrasonography in diagnosis of this syndrome. Methods : Clinically normal adult cavalier King Charles spaniel underwent ultrasonographic examination of the spinal cord and caudal fossa. Computed tomography was used to measure the caudal fossa and magnetic resonance imaging allowed syringomyelia and cerebellar herniation identification. Results : Of the 16 dogs in the study, seven had syringomyelia (43·7 per cent). All dogs had cerebellar herniation, suggesting Chiari‐like malformation and also a tendency to occipital dysplasia. Computed tomography measurements of the caudal fossa are reported. In one dog, a syrinx was identified by ultrasonography. The only difference between dogs with or without syringomyelia was that dogs with Chiari‐like malformation/syringomyelia were statistically older. Clinical Significance : The incidence of Chiari‐like malformation and syringomyelia may be high in an asymptomatic population of cavalier King Charles spaniel . Computed tomography measurements reported in this study should now be compared with those of a symptomatic population to evaluate the hypothesis that dogs with Chiari‐like malformation/syringomyelia syndrome have a smaller caudal fossa. This study did not identify a smaller caudal fossa in an asymptomatic cavalier King Charles spaniel population with syringomyelia. Ultrasonography probably has a low sensitivity for diagnosis of Chiari‐like malformation/syringomyelia.     

Comparison of cerebral cranium volumes between cavalier King Charles spaniels with Chiari-like malformation, small breed dogs and Labradors

O bjectives : To ascertain whether cavalier King Charles spaniels (CKCSs) have a proportionately smaller caudal fossa compared with other small dogs and with Labradors. To evaluate if cerebellar herniation in CKCS correlates with caudal fossa volume.
M ethods : In this retrospective study, three-dimensional images were created from magnetic resonance imaging brain series of 117 dogs (split into three groups: CKCS, Labradors and small breeds) from which the volumes of the fossae and brain parenchyma were calculated. These volumes were transformed into percentages of total cranial cavity and parenchyma volumes, respectively. The percentages were statistically compared among the groups. The percentage of herniated cerebellum in the CKCS was compared using linear regression with the caudal fossa and parenchyma percentages.
R esults : Cavalier King Charles spaniels had a proportionately smaller caudal fossa compared with Labradors (P=0·002) but not to small breeds (P=0·103). Their caudal fossa parenchyma was proportionately the same volume as Labradors (P=0·976) but greater than small breeds (P=0·005). No relationship was found for the per cent of cerebellum herniated.
C linical S ignificance : The results support mesoderm insufficiency or craniosynostosis as the pathogenesis of Chiari-like malformation (CM) in CKCS. It presents evidence for overcrowding of the caudal fossa due to a mismatch of brain parenchyma and fossa volumes as to why CKCS and not other small dogs are affected.     

Influence of head positioning on the assessment of Chiari-like malformation in Cavalier King Charles spaniels

Chiari-like malformation (CM) is almost omnipresent in the Cavalier King Charles spaniels (CKCS), often leading to syringomyelia (SM). Morphometric studies have produced variable results concerning relationship between the brain parenchyma within the caudal cranial fossa (CCF) and SM. The present study assesses the effect of head position, one potential confounder. Magnetic resonance images of CKCS with CM were reviewed in extended and flexed head positions. Volumes were calculated from transverse T2-weighted brain images. Mid-sagittal images were used for measurement of cerebellar herniation and CSF space between cerebellum and brainstem. Fourteen CKCS were included into the study, seven dogs with CM and seven with CM/SM. There was no difference between the relative brain parenchyma within the CCF in extended position and flexed position, or the brain parenchyma within the rostral and middle cranial fossae proportion. Cerebellar herniation and CSF space between cerebellum and brainstem were significantly increased in the flexed position. Cerebellar herniation and CSF space differed significantly between CM and CM/SM in a flexed head position. Volumetric measurements did not vary with head position. Cerebellar herniation and CSF space between the cerebellum and the brainstem were larger in a flexed head position.     

Syringomyelia in cavalier King Charles spaniels: the relationship between syrinx dimensions and pain

OBJECTIVES: This study was designed to test the hypothesis that pain associated with syringomyelia in dogs is dependent upon size and involvement of the dorsal part of the spinal cord. METHODS: Masked observers determined syrinx dimensions and precise location within the spinal cord on magnetic resonance images of 55 cavalier King Charles spaniels with syringomyelia. After removal of masking, syrinx size and location were compared between the cohorts of dogs that exhibited pain with those that did not. RESULTS: Maximum syrinx width was the strongest predictor of pain, scratching behaviour and scoliosis in dogs with syringomyelia. Both pain and syrinx size were positively correlated with syrinxes located in the dorsal half of the spinal cord. CLINICAL SIGNIFICANCE: Large syrinxes associated with damage to the dorsal part of the spinal cord are associated with persistent pain suggesting that the pain behaviour expressed by this group of patients is likely to be "neuropathic pain," resulting from disordered neural processing in the damaged dorsal horn. As such it is likely that conventional analgesic medication may be ineffective.     

Evaluation of the Swedish breeding program for cavalier King Charles spaniels

A breeding program with the aim of reducing the prevalence of mitral regurgitation (MR) caused by myxomatous mitral valve disease (MMVD) in Cavalier King Charles Spaniels (CKCS) is currently ongoing in Sweden. In this investigation 353 CKCS were selected as a sample of the population and 150 were examined by auscultation for heart murmurs when they reached the age of six years in 2007 and 2009. The aim with this investigation was to study the prevalence of heart murmurs in six-year-old CKCS and to estimate if prevalence has decreased since the breeding program was introduced 2001. The effect of the breeding program was evaluated by comparing the prevalence of heart murmurs in the two groups. In 2007, the prevalence of heart murmurs was 52% (50% for females and 54% for males) and in 2009, the prevalence was 55% (44% for females and 67% for males). No significant difference was found in the prevalence of heart murmurs between 2007 and 2009 (P = 0.8). For all six-year-old CKCS, the prevalence of heart murmur was 53% (females 46% and males 61%), which is higher than previous Swedish investigations.     

Hereditary aspects of occipital bone hypoplasia and syringomyelia (Chiari type I malformation) in cavalier King Charles spaniels

A database of over 1300 cavalier King Charles spaniels spanning 20 generations was established by obtaining pedigree information from 45 dogs with syringomyelia secondary to occipital bone hypoplasia. These data were supplemented with published information from the breed club. The incidence of syringomyelia was very high in certain families and lines which had been extensively inbred. The affected dogs could be traced back to one bitch born in 1956 and the two offspring from her single litter. Four key dogs representing four major breeding lines consistently occurred within the individual pedigrees. If a dog had more than five of its eight great-grandparents descended from these four lines there was a greater chance of it having syringomyelia. The data from this preliminary study suggest that occipital bone hypoplasia is hereditary in the cavalier King Charles spaniel and that its inheritance is more likely to be autosomal recessive because both dam and sire must be inbred descendants from certain lines. However, the inheritance is more likely to be of variable penetrance or oligogenic than simple.     

Reader performance in radiographic diagnosis of signs of mitral regurgitation in cavalier King Charles spaniels

O bjectives : To measure accuracy and variability of diagnosis by radiography of heart enlargement (HE) and heart failure (HF) in mitral regurgitation (MR).
M ethods : Sixteen readers representing four levels of experience evaluated 50 sets of radiographs with varying severity of MR for presence or absence of HE, left atrial enlargement (LAE) and HF. The performance of the readers was compared with a reference standard, using area under the curve (AUC) of receiver operating characteristic (ROC) curves. The interreader agreement value kappa (K) was calculated. A subset of difficult cases of HF was analysed before and after removing an outlying reader from each group.
R esults : AUC for HE was 0·89, for LAE it was 0·93 and for HF it was 0·92. Experience increased certainty of diagnosis but not accuracy. K ranges were HE, 0·53 to 0·67; LAE, 0·61 to 0·69 and HF, 0·49 to 0·58. When only difficult cases of HF were read, accuracy decreased and experienced readers performed better than inexperienced. When outlying readers were excluded, the differences between experienced and inexperienced readers increased.
C linical S ignificance : LAE, not HE, should be used to evaluate the heart size and indirectly the severity of MR on radiographs. For HF, agreement among individual readers was only moderate. Studies of reader accuracy should consider the effects of interreader variability.     

Echocardiographic mitral valve prolapse in cavalier King Charles spaniels: epidemiology and prognostic significance for regurgitation

This study investigated the epidemiology and prognostic significance of mitral valve prolapse, detected by ultrasonography, in 153 cavalier King Charles spaniels which were screened consecutively during a period of one year. Seventy-five of the dogs, which had either no murmur or a grade I murmur on screening, were reexamined three years later. The screening revealed that 82 per cent of the dogs aged one to three years and 97 per cent of the dogs over three years had various degrees of mitral valve prolapse. The presence and severity of the condition were independent of gender but correlated positively with age and negatively with bodyweight. The degree of mitral valve prolapse at screening correlated with the regurgitation status (murmur intensity and size of the regurgitant jets) at re-examination and with the percentage increase in the left ventricular end diastolic diameter over the three-year period. The presence of a grade I murmur was not a useful prognostic indicator.     

Syringohydromyelia in Cavalier King Charles spaniels

Syringohydromyelia secondary to foramen magnum overcrowding is described in seven Cavalier King Charles spaniels. Clinical signs were consistent with a central spinal cord lesion. The most common signs were persistent scratching at the shoulder region with apparent neck, thoracic limb, or ear pain and thoracic limb lower motor neuron deficits. The diagnosis was made by magnetic resonance imaging. The syringohydromyelia is postulated to be a consequence of an occipital bone malformation resulting in a small caudal fossa and cerebellar herniation. Clinical signs improved but did not completely resolve when the dogs received treatment with corticosteroids or nonsteroidal anti-inflammatory drugs.     

Thrombocytopenia in the cavalier King Charles spaniel

Blood samples were collected for the determination of platelet counts from 102 cavalier King Charles spaniels which had no history of bleeding disorders. Thrombocytopenia was found in 31 per cent of the samples when the lower reference limit was set at 100 X 10⁹/litre. The platelet count of the males (mean ± SD, 147 X 10⁹/litre ± 15 X 10⁹/litre) was found to be significantly (P<0.05) lower than that of the females (mean ± SD, 202 X 10⁹/litre ± 13 X 10⁹/litre). The platelet counts from a reference group of 16 normal beagles, analysed identically, were not lower than 189 X 10⁹/litre and no significant difference was found between males (mean ± SD, 249 X 10⁹/litre ± 14 X 10⁹/litre) and females (mean ± SD, 282 X 10⁹/litre ± 20 X 10⁹/litre).     

Heritability of syringomyelia in Cavalier King Charles spaniels

Mixed model analysis of 384 Cavalier King Charles spaniels (CKCS), with a magnetic resonance imaging diagnosis for the presence or absence of a syrinx, in conjunction with the Kennel Club pedigree records of all dogs registered from the mid 1980s to September 2007, revealed a moderately high estimate of heritability of syringomyelia (h² = 0.37 ± 0.15 standard error) when analysed as a binary trait. Inspection of cases where the disease segregated within families pointed to genes at more than one locus influencing syringomyelia. The availability of estimated breeding values for Kennel Club registered CKCS is a significant step in being able to select against syringomyelia, particularly given the difficulty of ascertaining the disease phenotype.     

Relationship of brain parenchyma within the caudal cranial fossa and ventricle size to syringomyelia in cavalier King Charles spaniels

Objectives : To assess if the volumes of the caudal cranial fossa (CCF), parenchyma within the caudal cranial fossa (CCFP) or ventricles (V) are associated with syringomyelia (SM) in cavalier King Charles spaniels (CKCS) with Chiari-like malformation (CM). To evaluate if volumes are associated with transverse syrinx width. Methods : Magnetic resonance images of 59 CKCS with CM were retrospectively reviewed and grouped with or without SM. Three-dimensional images were created and volumes of the fossae, brain parenchyma and ventricular system were calculated from which percentages of CCF, CCFP and V were created. If present, syrinx size was measured from its maximal transverse width. The percentages were statistically compared between groups, and correlation between percentages and syrinx dimensions was made. Results : CKCS with SM had significantly higher CCFP (P=0·0001) and V (P=0·0002) to those without but no significant difference in CCF (P=0·925). There was a positive correlation between CCFP and syrinx width (Pearson r=0·437) and ventricle size to syrinx width (Spearman r=0·627). Clinical Significance : A more marked overcrowding of the CCF is associated with SM, which may explain the high incidence of SM in CKCS with CM. The association between ventricle and syrinx dimensions supports the theory that SM development is the result of altered cerebrospinal fluid dynamics.     

Neurological signs and results of magnetic resonance imaging in 40 cavalier King Charles spaniels with Chiari type 1-like malformations

In human beings a Chiari type 1 malformation is a developmental condition characterised by cerebellar herniation and syringohydromyelia. Abnormalities compatible with such a malformation were identified by magnetic resonance imaging in 39 cavalier King Charles spaniels with neurological signs and in one neurologically normal cavalier King Charles spaniel that was examined postmortem. The dogs with these abnormalities had a wide variety of neurological signs, but there was no apparent correlation between the neurological signs and the severity of cerebellar herniation, syringohydromyelia or hydrocephalus.