Interleukin-31 in serum and cerebrospinal fluid of dogs with syringomyelia.

BACKGROUND: Syringomyelia is a spinal cord cavity containing cerebrospinal fluid (CSF)-like fluid. If syringomyelia asymmetrically involves the dorsal horn grey matter of the spinal cord, affected dogs show increased signs of dysesthesia and neuropathic pain, like increased itching behaviour. In the dorsal horn, amongst others, receptors for Interleukin-31 (IL-31) can be found. IL-31 is one of the main cytokines involved in the pathogenesis of pruritus in atopic dermatitis in different species. This study investigates suspected elevated levels of IL-31 in serum and CSF of dogs showing signs of pain or increased itching behaviour related to syringomyelia. The IL-31 were measured in archived samples (52 serum and 35 CSF samples) of dogs with syringomyelia (n = 48), atopic dermatitis (n = 3) and of healthy control dogs (n = 11) using a competitive canine IL-31 ELISA. RESULTS: Mean serum IL-31 level in dogs with syringomyelia was 150.1 pg/ml (n = 39), in dogs with atopic dermatitis 228.3 pg/ml (n = 3) and in healthy dogs 80.7 pg/ml (n = 10). Mean CSF IL-31 value was 146.3 pg/ml (n = 27) in dogs with syringomyelia and 186.2 pg/ml (n = 8) in healthy dogs. Individual patients with syringomyelia (especially dogs with otitis media or otitis media and interna or intervertebral disc herniation) showed high IL-31 levels in serum and CSF samples, but the difference was not statistically significant. IL-31 serum and CSF levels did not differ significantly in dogs with syringomyelia with or without itching behaviour and with or without signs of pain. CONCLUSION: Based on this study, increased IL-31 levels seem not to be correlated with itching behaviour or signs of pain in dogs with syringomyelia, but might be caused by other underlying diseases.

Presacral neuroendocrine tumors associated with the Currarino syndrome.

Currarino syndrome (CS) is an autosomal dominant syndrome caused by mutations in MNX1 and characterized by anorectal abnormalities, partial sacral agenesis, and presacral masses. The presacral masses are typically benign; however, malignant degeneration can occur, and presacral neuroendocrine tumors (NETs) have been reported in six cases. We report three individuals from two families affected by CS in which multiple individuals developed presacral NETs. The first family, 491, had six members with features of CS, including two siblings who presented with presacral, Grade 2 NETs, one of which had metastasized to bone and lymph nodes. A germline c.874C>T (p.Arg292Trp) mutation was found in a highly conserved region of MNX1 in three affected members who underwent sequencing. A second somatic variant/deletion in MNX1 was not detected in either patient's tumor. In the second family, 342, the proband presented with an incidentally discovered presacral NET. The proband's father had previously undergone resection of a presacral NET, and so genetic testing was performed, which did not reveal an MNX1 mutation or copy number variants. The lack of a second, somatic mutation in the tumors from family 491 argues against MNX1 acting as a tumor suppressor, and the absence of a germline MNX1 mutation in family 342 suggests that other genetic and anatomic factors contribute to the development of presacral NETs. These cases highlight the variable presentation of CS, and the potential for malignancy in these patients.

Nonreflecting Boundary Conditions for a CSF Model of Fourth Ventricle: Spinal SAS Dynamics.

In this paper, we introduce a one-dimensional model for analyzing the cerebrospinal fluid dynamics within the fourth ventricle and the spinal subarachnoid space (SSAS). The model has been derived starting from an original model of Linninger et al. and from the detailed mathematical analysis of two different reformulations. We show the steps of the modelization and the rigorous analysis of the first-order nonlinear hyperbolic system of equations which rules the new CSF model, whose conservative-law form and characteristic form are required for the boundary conditions treatment. By assuming sub-critical flows, for the particular dynamics we are dealing with, the most desirable option is to employ the nonreflecting boundary conditions, that allow the simple wave associated with the outgoing characteristic to exit the computational domain with no reflections. Finally, we carry out some numerical simulations related to different cerebral physiological conditions.

Intrathecal Morphine in the Presence of a Syrinx in Pediatric Spinal Deformity Surgery.

BACKGROUND: Intrathecal morphine (IM) is a popular adjunct for pain relief during pediatric spinal deformity surgery. There is no literature regarding its usefulness and safety in the presence of a spinal cord syrinx for patients undergoing spinal instrumentation. Anesthesiologists have previously been reluctant to use IM in the presence of any syrinx. METHODS: We retrospectively reviewed all patients with a preoperatively diagnosed spinal cord syrinx undergoing spinal deformity surgery who received IM and did not receive IM (non-IM). We recorded location of the syrinx, surgical time, length of stay, unexpected pediatric intensive care unit (PICU) admission, IM related complications (neurological, respiratory depression, or pruritus, nausea/vomiting), and reason for no IM administration. Patients with a syrinx and myelodysplasia (8), tethered spinal cord (4), paraplegia (1), holocord (1), neuroblastoma (1), and spinal cord glioma (1) were not given IM. Other reasons included a failed attempt (1), expectedly short surgical time (1), and anesthesiologist declined (2). RESULTS: There were 42 patients who met the inclusion criteria. Twenty-two patients received IM, while 20 patients did not. Patients receiving IM had 4 cervical, 5 cervicothoracic, 12 thoracic syrinxes, and 1 holocord syrinx. The non-IM group had 8 cervicothoracic, 6 thoracic, 4 holocord syrinxes, and 2 had unclassified locations. There were no neurological complications in the IM group, and 1 patient experienced respiratory depression following a shorter than expected surgery and was observed overnight in the PICU. One patient in the non-IM group with a holocord syrinx had temporary lower extremity weakness postoperatively that completely resolved and 4 patients were unexpectedly admitted to the PICU. Pruritus and nausea/vomiting was mild and similar in both groups. CONCLUSIONS: Our study demonstrates that with careful preoperative evaluation, most patients with a spinal cord syrinx can safely be given IM. Certain patients, such as those with a spinal holocord syrinx may have anatomic reasons to avoid IM, but those who are deemed appropriate for IM can receive it safely. LEVEL OF EVIDENCE: Level III-therapeutic study; retrospective comparative study.

Acute deterioration of adults with Chiari I malformation associated with extensive syrinx.

Background: Chiari I malformation (CMI) is usually a chronic disorder, although it may also present as a life-threatening condition with need for urgent decompression. Studies of acute deterioration of CMI are scarce. The identification of preoperative radiological factors that predict acute deterioration in adult patients with CMI facilitates planning of early decompression and decreases CMI-related morbidity.Patients and methods: This is a retrospective study of all adult patients with CMI who were referred to Sahlgrenska University Hospital, Sweden and underwent occipitocervical decompression in the period 2006-2016. In total, 65 consecutive patients were included and data regarding the preoperative degree of tonsillar herniation and syrinx (size, length and degree of rostral extension) were registered. Acute deterioration and the need for urgent surgical decompression within 24 h of hospitalisation were noted.Results: Three patients presented with acute deterioration of symptoms (4.6%) and showed an increased length and size of syrinx and more rostral extension of syrinx as compared to non-acute patients with CMI.Conclusion: Our study highlights the incidence of acute deterioration of CMI and shows the importance of assessing preoperative size, length and rostral extension of CMI-associated syrinx as indicators of acute deterioration so that CMI patients can be assigned to early surgical decompression.

Comparison of high-field MRI and multidetector CT for grading Chiari-like malformation and syringomyelia in Cavalier King Charles Spaniels.

Chiari-like malformation (CM) and syringomyelia (SM) are common illnesses that can cause debilitating neuropathic pain in Cavalier King Charles spaniels (CKCS). The current imaging modality to screen CKCS for CM/SM is MRI of the brain and cervical spine. Magnetic resonance imaging provides good soft tissue detail and contrast of the cerebellum and cervical spinal cord. Computed tomography (CT) is another cross-sectional imaging technique that facilitates brain and neck evaluation; however, soft tissue resolution does not match that of MRI. Computed tomography benefits include identification of concurrent craniocervical junction anomalies (atlantooccipital overlap) and shorter imaging/anesthesia times with the ability to use only sedation. The aim of this retrospective, method comparison study is to assess the utility of multidetector CT for screening CM and SM in CKCS as compared to high-field MRI. Three groups of observers with different levels of experience graded CM and SM based on the British Veterinary Association/Kennel Club CM/SM classification criteria. Thirty CKCS underwent multidetector CT and 3 Tesla MRI studies. Computed tomography and MRI studies were reviewed at different timepoints to minimize bias. Computed tomography has lower Cohen's Kappa agreement for each observer group compared to MRI. The intraclass correlation coefficient averaging CM and SM for all groups was excellent using MRI, while CT was poor for SM and moderate for cerebellar herniation. Greater observer experience resulted in a higher agreement for CT and MRI. Magnetic resonance imaging should remain the standard for screening of CM and SM as CT can result in misclassification and greater disagreement.

Association between craniovertebral junction abnormalities and syringomyelia in patients with chiari malformation type-1.

OBJECTIVE: To assess the correlation between craniovertebral junction (CVJ) abnormalities and syringomyelia in patients with Chiari malformation type-1 (CM1). METHODS: This was a retrospective study including patients with CM1. Identification of cases was done by searching a radiology database at a university hospital from 2012 to 2017. Patients were divided into 2 groups based on whether CVJ abnormalities were present (CVJ+) or absent (CVJ-). The patients` demographic and clinical data were reviewed. All magnetic resonance imaging studies were examined by a certified neuroradiologist. RESULTS: Sixty-four consecutive patients with CM1 were included. The mean age was 24+/-17 years; 59% were females. The CVJ+ group had more female patients (p=0.012). The most frequent CVJ abnormality was platybasia (71%), followed by short clivus (44%) and cervical kyphosis (33%). The CVJ abnormalities were more in Syringomyelia cases (p=0.045). However, the results were not significant when hydrocephalus cases were excluded. CONCLUSION: Among CM1 patients, CVJ abnormalities were found more in patients with syringomyelia. Future studies with larger sample size are required to further study the correlation between CVJ abnormalities and both syringomyelia and hydrocephalus in CM1 patients.

Arachnoiditis ossificans associated with syringomyelia: a case report.

The association of arachnoiditis ossificans with syringomyelia is a rare pathological entity. We present an unusual case who presented with progressive myelopathy caused by arachnoidits ossificans and syringomyelia. The pathophysiology and treatment strategy of this rare entity are still controversial.

Carcinoid transformation of presacral dermoid cyst in patient with currarino syndrome: a case report.

Currarino syndrome (CS) is a congenital disorder characterized by partial sacral agenesis, anorectal malformation and a presacral mass. Only three cases of carcinoid transformation of the presacral mass have been described in the literature. We present a case of carcinoid transformation of presacral dermoid cyst in patient with Currarino syndrome.

Syringomyelia-like syndrome in neuromyelitis optica spectrum disorder complicated with Sjogren's syndrome: a case report.

BACKGROUND: Besides CSF-flow obstruction, syringomyelia is associated with inflammatory spinal cord lesions. However, syringomyelia-like syndrome concomitant with neuromyelitis optica spectrum disorder (NMOSD) and primary Sjogren's syndrome (pSS) is extremely rare. Here, we would like to report a case of a patient with syringomyelia-like syndrome in NMOSD complicated with Sjogren's Syndrome. CASE PRESENTATION: A 64-old male Han Chinese, presented with three episodes of acute demyelinating processes in the central nervous system within 5 years. Firstly, he presented with ascending left lower extremity weakness and numbness, and initially progressive loss of vision in the right eye before 5 years, and subsequently in the right eye 2 months later. High dose corticosteroid therapy was prescribed for this attack. Second, he suffered from refractory gastrointestinal symptoms. such as nausea, vomiting, abdominal pain and early satiety. After the second episode, he received long-term azathioprine and prednisone treatment in low dosages. Six months before admission, he developed the lower back pain and numbness in lower limbs, and urinary incontinence. This time, he complained of acute onset of right lower limb paralysis, paresthesia and urinary incontinence. MRI of the spine revealed a syringomyelia extending from the C7 to T4 levels with serum positive anti-aquaporin-4 antibodies (AQP4-Ab) (indirect immunofluorescence on AQP4 transfected cells). he was serologically positive for both anti-Sjögren's syndrome-related antigen A and B antibodies and there was reduced salivary flow on scintigraphy. Lip salivary gland (LSG) biopsies were graded (grade four lymphocytic infiltration) according to the Chisholm and Mason classification system and by morphometric analysis. And finally, diagnosed as syringomyelia-like syndrome in NMOSD complicated with Sjogren's syndrome. CONCLUSIONS: Although extremely rare, This index patient highlights that syringomyelia could be associated with underlying NMOSD and pSS, and autoimmune disorders should be considered in the initial differential diagnosis, This is very helpful for the therapeutic implications and evaluating curative effect.

Computer simulation of syringomyelia in dogs.

BACKGROUND: Syringomyelia is a pathological condition in which fluid-filled cavities (syringes) form and expand in the spinal cord. Syringomyelia is often linked with obstruction of the craniocervical junction and a Chiari malformation, which is similar in both humans and animals. Some brachycephalic toy breed dogs such as Cavalier King Charles Spaniels (CKCS) are particularly predisposed. The exact mechanism of the formation of syringomyelia is undetermined and consequently with the lack of clinical explanation, engineers and mathematicians have resorted to computer models to identify possible physical mechanisms that can lead to syringes. We developed a computer model of the spinal cavity of a CKCS suffering from a large syrinx. The model was excited at the cranial end to simulate the movement of the cerebrospinal fluid (CSF) and the spinal cord due to the shift of blood volume in the cranium related to the cardiac cycle. To simulate the normal condition, the movement was prescribed to the CSF. To simulate the pathological condition, the movement of CSF was blocked. RESULTS: For normal conditions the pressure in the SAS was approximately 400 Pa and the same applied to all stress components in the spinal cord. The stress was uniformly distributed along the length of the spinal cord. When the blockage between the cranial and spinal CSF spaces forced the cord to move with the cardiac cycle, shear and axial normal stresses in the cord increased significantly. The sites where the elevated stress was most pronounced coincided with the axial locations where the syringes typically form, but they were at the perimeter rather than in the central portion of the cord. This elevated stress originated from the bending of the cord at the locations where its curvature was high. CONCLUSIONS: The results suggest that it is possible that repetitive stressing of the spinal cord caused by its exaggerated movement could be a cause for the formation of initial syringes. Further consideration of factors such as cord tethering and the difference in mechanical properties of white and grey matter is needed to fully explore this possibility.

Clinical manifestations and radiological characteristics in patients with idiopathic syringomyelia and scoliosis.

PURPOSE: To clarify the clinical manifestation and radiological characteristics of idiopathic syringomyelia (IS) and to investigate the relationship between syrinx and scoliotic curves in IS-related scoliosis patients. METHODS: Fifty-five patients with IS and scoliosis were identified and reviewed retrospectively from June 2009 to December 2016. Radiographic features of syrinx, scoliosis and clinical manifestations of neurological deficits were collected. The syrinx/cord (S/C) ratio was defined as the anteroposterior diameter of syrinx divided by the diameter of spinal cord at the same level. Patients were classified into two groups, the thoracic group (T group, apex vertebra located from T2 to intervertebral disk of T11-T12) and the thoracolumbar/lumbar group (TL/L group, apex vertebra located from T12 to L5). RESULTS: There was no correlation between the radiological features of idiopathic syrinx and scoliotic curve parameters. The TL/L group had a lower level of most caudal extent (13.7 compared with 10.6, P = 0.029) and lower level of largest S/C ratio (12.0 compared with 8.7, P = 0.016) than that in T group. The deviated side of syrinx was not coincident with major curve convexity (27.2% concordance rate, P = 0.522) or dominant side of neurological deficit (16.3% concordance rate, P = 0.212). CONCLUSIONS: Patients with major curves located on the thoracolumbar or lumbar spine had a much lower caudal extent and lower level of greatest S/C ratio compared to patients with major curves located on the thoracic spine. No significant relationships were detected between syrinx features, scoliotic curve parameters and neurological deficits. These slides can be retrieved under Electronic Supplementary Material.

Thoracic ossification of ligamentum flavum manifesting holocord syringomyelia: case report.

It has been reported that syringomyelia is rarely associated with degenerative spinal disorders, but the case of holocord syringomyelia is never reported. We here present a case of a 59-year-old woman with right shoulder pain, dysesthesia of the right hand, and gait disturbance. Radiographically, examinations of the spine demonstrated holocord syringomyelia with ossification of ligamentum flavum at T2/3 level. Holocord syringomyelia was reduced remarkably after posterior decompression at the T2/3 level, and her symptoms also improved. We speculated that holocord syringomyelia might have developed due to craniospinal pressure dissociation caused by focal compression of dural sac from extradural degenerative change.

The 3D characteristics of post-traumatic syringomyelia in a rat model: a propagation-based synchrotron radiation microtomography study.

Many published literature sources have described the histopathological characteristics of post-traumatic syringomyelia (PTS). However, three-dimensional (3D) visualization studies of PTS have been limited due to the lack of reliable 3D imaging techniques. In this study, the imaging efficiency of propagation-based synchrotron radiation microtomography (PB-SRµCT) was determined to detect the 3D morphology of the cavity and surrounding microvasculature network in a rat model of PTS. The rat model of PTS was established using the infinite horizon impactor to produce spinal cord injury (SCI), followed by a subarachnoid injection of kaolin to produce arachnoiditis. PB-SRµCT imaging and histological examination, as well as fluorescence staining, were conducted on the animals at the tenth week after SCI. The 3D morphology of the cystic cavity was vividly visualized using PB-SRµCT imaging. The quantitative parameters analyzed by PB-SRµCT, including the lesion and spared spinal cord tissue area, the minimum and maximum diameters in the cystic cavity, and cavity volume, were largely consistent with the results of the histological assessment. Moreover, the 3D morphology of the cavity and surrounding angioarchitecture could be simultaneously detected on the PB-SRµCT images. This study demonstrated that high-resolution PB-SRµCT could be used for the 3D visualization of trauma-induced spinal cord cavities and provides valuable quantitative data for cavity characterization. PB-SRµCT could be used as a reliable imaging technique and offers a novel platform for tracking cavity formation and morphological changes in an experimental animal model of PTS.

MRI characteristics for "phantom" scratching in canine syringomyelia.

BACKGROUND: A classic sign of canine syringomyelia (SM) is scratching towards one shoulder. Using magnetic resonance imaging (MRI) we investigate the spinal cord lesion relating to this phenomenon which has characteristics similar to fictive scratch secondary to spinal cord transection. Medical records were searched for Cavalier King Charles spaniels with a clinical and MRI diagnosis of symptomatic SM associated with Chiari-like malformation (CM). The cohort was divided into SM with phantom scratching (19 dogs) and SM but no phantom scratching (18 dogs). MRI files were anonymised, randomised and viewed in EFILM ™. For each transverse image, the maximum perpendicular dimensions of the syrinx in the dorsal spinal cord quadrants were determined. Visual assessment was made as to whether the syrinx extended to the superficial dorsal horn (SDH). RESULTS: We showed that phantom scratching appears associated with a large dorsolateral syrinx that extends to the SDH in the C3-C6 spinal cord segments (corresponding to C2-C5 vertebrae). Estimated dorsal quadrant syrinx sizes based on the perpendicular diameters were between 2.5 and 9.5 times larger in dogs with phantom scratching, with the largest mean difference p-value being 0.009. CONCLUSION: SM associated phantom scratching appears associated with MRI findings of a large syrinx extending into the mid cervical SDH. We hypothesise that damage in this region might influence the lumbosacral scratching central pattern generator (CPG). If a scratching SM affected dog does not have a large dorsolateral cervical syrinx with SDH involvement then alternative explanations for scratching should be investigated.

Pathological findings in low-field magnetic resonance imaging of the canine spine - a study of 112 patients.

Magnetic resonance imaging (MRI) is a method of choice in diagnosing nervous system disorders. This paper presents the results of a study where selected segments of the canine spine were examined by low-field MRI in 112 patients. Images of pathological changes were obtained in spin echo (SE), fast spin echo (FSE) and hybrid contrast enhancement (3D HYCE) sequences. The cervical region of the spinal cord (C1-C5) was examined in 32 patients, the cervicothoracic region (C6-Th2)- in 14 patients, the thoracolumbar region (Th3-L3) - in 23 patients, and the lumbosacral region (L4-S3) - in 43 patients. The results were used to determine the incidence of pathological changes in different sections of the canine spine, such as intervertebral disc disease (IDD), disc desiccation, syringomyelia and changes characterized by higher uptake of the contrast medium. Intervertebral disc disease was diagnosed in 52.7% of patients and it was the most common abnormality. Disc dehydratation without protrusion or extrusion was noted in 23.2% of animals. Pathological changes with increased uptake of the contrast medium and indicative of neoplastic growth were observed in 13.4% of patients and syringomyelia was diagnosed in 9.82% of the examined animals. The proposed sequences revealed the presence of above abnormalities.

Comparison of the scoliosis curve patterns and MRI syrinx cord characteristics of idiopathic syringomyelia versus Chiari I malformation.

PURPOSE: Although the more readily available MR imaging has brought about more incidental findings of idiopathic syringomyelia (IS), no published study has specifically addressed the clinical and imaging features of IS-associated scoliosis. Since IS and Chiari I malformation (CMI)-type syringomyelia are hypothesized to share a common underlying developmental pathomechanism, this study aimed to investigate the scoliosis curve patterns and MRI syrinx cord characteristics of patients with IS comparing with those seen in CMI. METHODS: Sixty-one patients with scoliosis secondary to IS were identified and reviewed retrospectively. The curve pattern and specific curve features were recorded and compared with historic CMI controls. Location, size, and morphological appearance of the syrinx were systematically assessed on MR images. RESULTS: The maximal syrinx/cord ratio and rostrocaudal length of the syrinx in IS averaged 0.43 ± 0.16 (range 0.17-0.78) and 4.6 ± 2.5 (range 2-15) vertebral levels, respectively, both of which were smaller than those reported in CMI-type syringomyelia. Regarding the characteristics of IS-related scoliosis, sagittal profiles as well as the frequency of curve patterns and atypical features were all found to resemble those in patients with CMI (P > .05). Among the 47 individuals with a single thoracic curve, Fisher exact test revealed a significant correlation between curve convexity and the dominant side of deviated syrinx (83.3 % concordance rate, P = .021). In addition, apex of the thoracic curve trended toward being significantly correlated with the level of maximum expansion of the syrinx (P = .066). CONCLUSIONS: Radiological characteristics of scoliosis were found to be similar between idiopathic and CMI-type syrinx in both the coronal and sagittal planes, adding further evidence to the concept that these entities may be part of a spectrum of disease sharing a common pathophysiological mechanism. The thoracic spine in IS patients tended to be convex to the deviated side of syrinx, which indirectly supported the likely role of spinal cord dysfunction in the pathogenesis of syrinx-associated spinal deformities.

Morphometric features of posterior cranial fossa are different between Chiari I malformation with and without syringomyelia.

PURPOSE: To investigate whether the posterior cranial fossa (PCF) morphology in Chiari I malformation without syringomyelia (also called syrinx) (CMI-only) is different from that in Chiari I malformation with syrinx (CMI-S). METHODS: Nineteen CMI patients without syrinx constituted the CMI-only group, whereas 48 CMI patients with syrinx were assigned to the CMI-S group. Another cohort of 40 age-matched asymptomatic adolescents was enrolled to serve as the control group. Six measurements were evaluated and compared between these three groups from T1-weighted magnetic resonance (MR) imaging, including the length of the clivus (AB), the anteroposterior diameter of the foramen magnum (BC), the length of the supraocciput (CD), the anteroposterior diameter of the posterior fossa (DA), the posterior fossa height (BE) and the clivus gradient ([Formula: see text]). The posterior cranial fossa morphology in relation to syrinx severity was also investigated. RESULTS: Compared to the normal controls, the AB, CD, DA, BE and [Formula: see text] were significantly larger in the CMI-S group. Similar changes in AB, CD, DA and BE were also demonstrated in the CMI-only group, while the clivus gradient ([Formula: see text]) was found to be normal when compared with the control group. A significantly decreased clivus gradient was observed in the CMI-S group as compared to CMI-only group. In addition, the clivus was significantly flattened in patients with a distended-syrinx in comparison to those with a non-distended syrinx. CONCLUSIONS: Small size of the posterior fossa was detected both in CMI cases with and without syrinx. The clivus gradient served as the only morphologic difference in the PCF between CMI-S and CMI-only patients and was correlated with the severity of the syrinx, may support the theory that the restricted circulation of cerebrospinal fluid at the anterior paramedial subarachnoid space contributes to the formation of a syrinx.

PROGRESSIVE SYRINGOHYDROMYELIA AND DEGENERATIVE AXONOPATHY IN A BOBCAT (LYNX RUFUS) FOLLOWING SURGICAL CORRECTION OF A CHIARI-LIKE MALFORMATION.

A 3-yr-old male captive bobcat (Lynx rufus) presented with chronic ataxia and right-sided head tilt. Magnetic resonance imaging (MRI) revealed cerebellar crowding and compression consistent with Chiari-like malformation. The clinical signs did not improve after surgical occipital craniectomy, and 2 mo postoperatively a second MRI showed hydromyelia and continued cerebellar compression. The bobcat was euthanized, and necropsy showed chronic focal cerebellar herniation and chronic multifocal atlanto-occipital joint osteophyte proliferation. Histology confirmed the presence of a thick fibrous membrane along the caudal aspect of the cerebellar vermis, suggestive of postoperative adhesions, and axonal degeneration of the cervical spinal cord, even in sections without a central canal lesion. These lesions appear to have been complications associated with surgical correction of the Chiari-like malformation.

[Results of surgical treatment of syringomyelia associated with Chiari 1 malformation. An analysis of 125 cases].

UNLABELLED: The rate of Chiari malformation (CM) in a population ranges from 3 to 8 per 100,000 population. In 62-80% of cases, CM is accompanied by the development of syringomyelia (SM) at various levels. The clinical picture in these patients is a combination of CM and SM manifestations; however, SM symptoms often prevail, which creates some problems in the diagnosis of the disease and in the choice of optimal treatment. OBJECTIVE: On the basis of our own experience of surgical interventions, we aimed to clarify the indications for surgical treatment of SM associated with CM and to determine the optimal amount of surgery and the criteria for evaluation of treatment outcomes. MATERIAL AND METHODS: Two hundred twenty five patients with a combination of syringomyelia and Chiari 1 malformation were examined in the period from 2011 to February 2015. Of them, 125 patients were operated on. The mean age of the operated patients was 56±8 years. The mean time from the appearance of the first signs of the disease to surgery was 75±82 months. All operations were performed by a single surgeon. The operations were carried out in the half-sitting (89.6%) or prone (10.4%) position. The operation included sparing suboccipital craniectomy, C1 arch resection, recovery of the cerebrospinal fluid (CSF) circulation along the posterior surface of the cerebellum, and reconstruction of the dura mater in the craniovertebral junction region. RESULTS: Exploration of the arachnoid mater of the cisterna magna after dura opening revealed no arachnopathy in 78 (62.4%) patients (Chiari 0 malformation according to Klekamp). The type 1 arachnopathy (by Klekamp) was detected in 31 patients (24.8%), and the type 2 arachnopathy was observed in 16 (12.8%). The condition of 109 (88%) patients was evaluated one year after the surgery. Sixty one (56%) patients had partial or complete regression of preoperative neurological symptoms. The disease stopped progressing in 44 patients (40%). The disease was progressing in 4 (3.7%) patients. No recurrence of a CSF circulation disturbance at the craniovertebral level was observed during follow-up. Early postoperative complications occurred in 4 (3.2%) patients: wound CSF leakage in 1 (0.8%) patient, acute epidural hematoma in 1 (0.8%) patient, and aseptic meningitis in 2 (1.6%) patients. Temporary deteriorations in the condition (headache worsening, meteosensitivity) were detected in 11 (8.9%) patients. These symptoms regressed by the end of the 1st postoperative month. There were no deaths. CONCLUSIONS: The indication for surgery in patients with a combination of CM and SM is the presence of neurological symptoms associated with syringomyelia and their progression as well as headache caused by herniation of the cerebellar tonsils, which significantly deteriorates the patient's quality of life. The main criteria for evaluating the efficacy of treatment include stabilization of the clinical symptoms and/or improvement in the patient condition. Suboccipital craniectomy followed by reconstruction of the dura mater and recovery of the CSF circulation in the craniovertebral region is an effective treatment of syringomyelia associated with Chiari 1 malformation.