Relationship between clinical parameters and malformations in dogs diagnosed with atlanto-axial instability.

Atlanto-axial instability is a common disease that affects toy-breed dogs. Most cases of atlanto-axial instability are congenital. Furthermore, patients with atlanto-axial instability are predisposed to other concurrent diseases. Therefore, this study aimed to retrospectively determine the presence of concurrent diseases in cases with atlanto-axial instability using imaging data and analyze the relationship between clinical parameters and the incidence of complex malformations. The clinical data and imaging findings of 41 toy-breed dogs diagnosed with atlanto-axial instability were analyzed using their medical records and imaging data. Occipital dysplasia (17/27), atlanto-occipital overlapping (22/34), dens dysplasia (27/41), Chiari-like malformation (8/34), syringomyelia (5/34), lateral ventricular enlargement (20/36), and intracranial arachnoid cyst (5/35) were observed in patients with atlanto-axial instability. The body weight of the patients in the groups with atlanto-occipital overlapping and lateral ventricular enlargement was lower than that of those in the groups without these diseases (1.78 ± 0.71 vs 2.71 ± 1.15 kg, P = 0.0269, 1.60 ± 0.40 vs 2.75 ± 1.08 kg, P = 0.001, respectively). Furthermore, when the correlation between the total number of concurrent diseases and the age at onset and body weight was examined, it became clear that lower body weight was associated with the incidence of a greater number of concurrent diseases. Thus, the findings of this study suggest that toy-breed dogs are more likely to present with complex malformations and should be evaluated carefully with additional examinations and treatment methods.

Epidemiology of Chiari I Malformation and Syringomyelia.

Chiari I malformation is a common condition seen by adult and pediatric neurosurgeons. With increased utilization of MRI over time, incidental findings of Chiari I malformation are occurring more frequently. The prevalence of symptomatic Chiari I malformation is much smaller than that of asymptomatic Chiari I malformation. The prevalence of Chiari I malformation-associated syringomyelia is likely overestimated in the literature. The epidemiology of Chiari I malformation and associated syringomyelia differs based on age, sex, ethnicity, race, and socioeconomic status. The natural history of Chiari I malformation and associated syringomyelia appears to be quite benign as few patients who are managed nonsurgically later require surgical intervention.

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

The objective of this study was to calculate lateral ventricles dimension in Cavalier King Charles Spaniel dogs with Chiari-like malformation and investigate the association between ventriculomegaly and signalment, clinical signs, ventricular asymmetry, grade of Chiari-like malformation, syringomyelia and index of medullary kinking. Retrospectively, 43 client-owned Cavalier King Charles Spaniels, older than 1 year of age, with magnetic resonance imaging diagnosis of Chiari-like malformation were enrolled. Initial and follow-up (up to 36 months) clinical status was graded. Images were reviewed to quantify the enlargement of lateral ventricles, evaluate ventricular symmetry, grade of Chiari-like malformation, grade of syringomyelia and medullary kinking index. Cases presenting epileptic seizures during the evaluation period were also recorded. The most common initial clinical signs were scratching and neck pain. Ventriculomegaly was identified in 70% of dogs, Chiari-like malformation grade 2 was observed in 77% of cases, ventricular asymmetry and syringomyelia were identified in 54% and 80% of dogs, respectively; the median medullary kinking index was 37.77%. Moreover, 28% of dogs presented epileptic seizures. No significant association was identified between dimension of lateral ventricles and signalment, clinical signs, and imaging findings; no significant association was identified between ventriculomegaly and epilepsy (P≥0.05). In conclusion, the prevalence of ventriculomegaly in Cavalier King Charles Spaniels is high but this finding does not seem related to the severity of clinical signs, presence of Chiari-like malformation, syringomyelia and craniocervical junction abnormalities such as medullary kinking.

Longitudinal scoliosis behavior in Chiari malformation with and without syringomyelia.

OBJECTIVE: The objective of this study was to understand the natural history of scoliosis in patients with Chiari malformation type I (CM-I) with and without syringomyelia. METHODS: A retrospective review of data was conducted. Patients with CM-I were identified from a cohort of 14,118 individuals age 18 years or younger who had undergone MRI over an 11-year period at the University of Michigan. Patients eligible for study inclusion had a coronal curve ≥ 10° on radiography, associated CM-I with or without syringomyelia, and at least 1 year of clinical follow-up prior to any surgery. Curve magnitude at initial diagnosis, prior to posterior fossa decompression (PFD; if applicable), and at the last follow-up (prior to any surgical correction of scoliosis) was recorded, and clinical and radiographic characteristics were noted. The change in curve magnitude by 10° was defined as curve progression (increase by 10°) or regression (decrease by 10°). RESULTS: Forty-three patients met the study inclusion criteria and were analyzed. About one-third (35%) of the patients presented with symptoms attributed to their CM-I. The mean degree of scoliosis at presentation was 32.6° ± 17.7°. Twenty-one patients (49%) had an associated syrinx. The mean tonsil position below the level of the foramen magnum was 9.8 ± 5.8 mm. Patients with a syrinx were more likely to have a curve > 20° (86% vs 41%, p = 0.002). Curve magnitude remained stable (≤ ±10°) in 77% of patients (33/43), progressed in 16% (7/43), and regressed in 7% (3/43). Mean age was higher (14.8 ± 0.59 years) among patients with regressed curves (p = 0.026). All regressed curves initially measured ≤ 20° (mean 14° ± 5.3°), and none of the patients with regressed curves had a syrinx. The change in curve magnitude was statistically similar in patients with (7.32° ± 17.7°) and without (5.32° ± 15.8°) a syrinx (p = 0.67). After a mean follow-up of 3.13 ± 2.04 years prior to surgery, 27 patients (63%) ultimately underwent posterior fossa or scoliosis correction surgery. For those who eventually underwent PFD only, the rate of change in curve magnitude prior to surgery was 0.054° ± 0.79°. The rate of change in curve magnitude was statistically similar before (0.054° ± 0.79°) and after (0.042° ± 0.33°) surgery (p = 0.45) for patients who underwent PFD surgery only. CONCLUSIONS: The natural history of scoliosis in the presence of CM-I is variable, though most curves remained stable. All curves that regressed were ≤ 20° at initial diagnosis, and most patients in such cases were older at scoliosis diagnosis. Patients who underwent no surgery or PFD only had similar profiles for the change in curve magnitude, which remained relatively stable overall, as compared to patients who underwent PFD and subsequent fusion, who demonstrated curve progression. Among the patients with a syrinx, no curves regressed, most remained stable, and some progressed. Understanding this variability is a first step toward building a prediction model for outcomes for these patients.

Spinal-Shortening Process Positively Improves Associated Syringomyelia in Patients with Scoliosis After Single-Stage Spinal Correction.

OBJECTIVE: Single-stage spinal correction without previous neurosurgical intervention has been attempted in patients with scoliosis associated with syringomyelia (SM). However, evidence to demonstrate its potential influence on associated SM from direct spinal correction is still lacking. The aim of the present study was to explore the role of spinal shortening in the prognosis of SM-associated scoliosis after single-stage spinal correction without previous neurosurgical intervention. METHODS: Patients with SM-associated scoliosis without previous neurological intervention, who had undergone posterior direct instrumental correction (PDIC) without osteotomy and posterior vertebral column resection (PVCR) at a single center, were selected for comparative analysis. The basic demographic and pre- and postoperative imaging data of the spinal deformity and SM at the final follow-up were compared separately for the 2 different spinal correction procedures. RESULTS: A total of 23 patients were included in the final analysis: 13 had undergone PDIC and 10 had undergone PVCR. The mean follow-up period was 6.2 years (range, 5-9 years). At the final follow-up, the mean corrective rate of scoliosis and kyphosis was 65.7% and 48.4%, respectively. Obvious SM reduction was achieved in 11 patients (47.8%), with an average reduction of 37.3%. No patient experienced neurologic deterioration or had required further neurosurgical intervention for SM during follow-up. The patients who had undergone PVCR had had much more severe scoliosis (98.8° vs. 60.5°; P = 0.000) and kyphosis (74.8° vs. 43.6°; P = 0.032). Moreover, 80.0% of the patients who had undergone PVCR had experienced obvious SM improvement compared with 23.1% of those who had undergone PDIC (P = 0.007). CONCLUSIONS: The reduction of spinal cord tension is an important factor influencing SM improvement. As the most powerful spinal-shortening osteotomy, PVCR can effectively correct severe spinal deformities and improve associated SM. Single-stage posterior spinal correction can be a potential choice for selected patients with scoliosis and untreated SM using strict inclusion criteria, which will not only achieve safe spinal correction but could also steadily improve and stabilize SM.

Statistical Shape Analyses of the Brainstem in Chiari Patients.

AIM: To ascertain whether the brain stem?s shape differs in patients with syrinx and without syrinx in cases with Chiari malformation Type I (CM-1), relative to healthy controls. MATERIAL AND METHODS: Data on marked brainstem regions were obtained from 2D digital image files. Generalized Procrustes analysis was used to evaluate shape differences among patients with syrinx, patients without syrinx, and healthy controls. Shape deformations among groups were examined by Thin Plate Spline (TPS) analysis. RESULTS: According to the brain stem shape, there were differences between patients with syrinx and controls, and between patients without syrinx and controls. High-level deformations were also observed among the groups. CONCLUSION: In the present study, the presence of shape deformations in Chiari patients? brainstem was demonstrated. This is the first study using a landmark-based geometrical morphometric method to demonstrate the shape difference in Chiari patients? brainstem.

Selective thoracolumbar/lumbar fusion for Syringomyelia-associated scoliosis: a case-control study with Lenke 5C adolescent idiopathic scoliosis.

BACKGROUND: Selective thoracolumbar/lumbar fusion technique was introduced to treat adolescent idiopathic scoliosis (AIS) patients with major thoracolumbar/lumbar curves. Theoretically, this surgical strategy could also be applied to syringomyelia patients. No previous study has specifically addressed the effectiveness of selective thoracolumbar/lumbar fusion for patients with syringomyelia-associated scoliosis. The aim of the study was to investigate the effectiveness of selective thoracolumbar/lumbar fusion for the surgical treatment of patients with syringomyelia-associated scoliosis. METHODS: From February 2010 to September 2016, 14 syringomyelia-associated patients with major thoracolumbar/lumbar curves were retrospectively reviewed. Besides, 30 Lenke 5C AIS patients were enrolled as a control group. Posterior selective thoracolumbar/lumbar fusion was performed for both groups. Patients' demographic, operative, radiological, and quality of life data were reviewed with follow-up. Intragroup comparisons were performed for each parameter. RESULTS: The two groups were matched by age, gender, curve characteristics, duration of follow-up, and all preoperative radiographic parameters except for thoracic kyphosis. After surgery, the average correction rate of the major thoracolumbar/lumbar curve was 82.2 ± 7.8% in the syringomyelia group, which was not significantly different from that of AIS group (82.5 ± 10.6%, P = 0.47). A similar improvement of unfused thoracic curve was observed between the two groups (50.1 ± 16.5% vs. 48.5 ± 26.9%, P = 0.29). During the follow-up, the correction effect of scoliosis was well maintained, without aggravation of the original neural symptoms or fresh permanent neurological deficits. Of note, the number of fusion levels was significantly larger in syringomyelia group than that in AIS group (7.6 ± 1.4 vs. 6.5 ± 1.2, P < 0.01). The average follow up was 47.6 months (36-81 months). CONCLUSION: Similar to AIS cases, syringomyelia-associated scoliosis can be effectively and safely corrected by selective thoracolumbar/lumbar fusion with satisfactory surgical outcomes. However, the syringomyelia group, on average, required an additional fused segment for treatment as compared to the AIS group (7.6 versus 6.5 in the AIS group).

Incidence of intraspinal abnormalities in congenital scoliosis: a systematic review and meta-analysis.

OBJECTIVE: This is the first systematic review and meta-analysis on the overall incidence of intraspinal abnormalities in patients with congenital scoliosis (CS) and potential influencing factors. METHODS: We searched three large electronic databases (PubMed, EMBASE, and Cochrane Library) for potentially relevant studies. The quality of the included studies was assessed independently by two authors using the Methodological Index for Non-Randomized Studies (MINORS) criteria. Data on the number of CS patients, number of CS patients with intraspinal abnormalities, sex of the patients, and CS types were extracted from the included studies. R software was used to pool and analyze all the extracted data. RESULTS: This meta-analysis included 10 articles, and 671 of 1863 CS patients undergoing magnetic resonance imaging (MRI) examinations were identified to have intraspinal abnormalities. The overall incidence of intraspinal abnormalities in the patients with CS was 37% (95% CI, 29-45%). Diastematomyelia was the most common intraspinal abnormality and was detected in 45.60% of the patients with intraspinal abnormalities (306/671). The remaining intraspinal abnormalities included syringomyelia (273/671, 40.69%), tethered cord (190/671, 28.32%), low conus (58/671, 8.64%), intraspinal mass (39/671, 5.81%), Chiari malformation (32/671, 4.77%), fatty filum (27/671, 4.02%), spina bifida (occulta excluded) (17/671, 2.53%), tumor (17/671, 2.53%), cyst (12/671, 1.79%), syringomyelus (4/671, 0.60%), dural ectasia (1/671, 0.15%), and undiagnosed cord MRI hyperintensity (1/671, 0.15%). The patient's sex and CS type were not factors that affected the incidence of intraspinal abnormalities in CS patients (all P > 0.05). CONCLUSIONS: This meta-analysis revealed that the overall incidence of intraspinal abnormalities detected by MRI in CS patients was 37%. Diastematomyelia was the most common intraspinal abnormality. The patient's sex and CS type were not factors that affected the incidence of intraspinal abnormalities in CS patients.

Neurological deficits are present in syndromic craniosynostosis patients with and without tonsillar herniation.

BACKGROUND: Children with syndromic craniosynostosis (sCS) have a higher incidence of cerebellar tonsillar herniation (TH) than the general population. In the general population, TH ≥ 5 mm below the foramen magnum is associated with typical neurological deficits but, in sCS, we do not know whether this degree of TH is required before such deficits occur. OBJECTIVE: This prospective cohort study aimed to determine the association between findings on neurological assessment and cerebellar tonsillar position. METHODS: Magnetic resonance imaging (MRI) was used to determine TH ≥ 5 mm and the presence of syringomyelia. In regard to the outcome of neurological deficits, these were categorized according to: A, cerebellar function; B, cranial nerve abnormalities; and C, sensory or motor dysfunction. RESULTS: Twenty of 63 patients with sCS (32% [95% confidence interval 21-45%]) had TH ≥ 5 mm and/or syringomyelia. There was no significant difference in proportion between individual forms of sCS: 16/34 Crouzon, 2/11 Muenke, 2/12 Apert, and 0/7 Saethre-Chotzen patients. Neurological deficits were prevalent (73% [95% confidence interval 60-83%]), and as frequent in patients with TH ≥ 5 mm and/or syringomyelia as those without. Surgery occurred in 3 patients overall, and only in Crouzon patients. CONCLUSION: Determining the effect of TH ≥ 5 mm on neurologic functioning in sCS patients is used to better determine when surgical intervention is warranted. However, we have found that neurological deficits are prevalent in sCS patients, irrespective of cerebellar tonsillar position, suggesting that such findings are developmental and, in part, syndrome-specific central nervous system features.

Syringomyelia and Chiari Syndrome Registry: advances in epidemiology, clinical phenotypes and natural history based on a North Western Italy cohort.

BACKGROUND: Syringomyelia and Chiari Syndrome are classified as rare diseases, but current known occurrence in Europe is missing. The increased ability to diagnose these pathologies by magnetic resonance imaging and its widespread availability has led to an increase of reported cases, often asymptomatic, with the need to standardize definitions, diagnostic criteria and treatments. AIMS: We present shared Interregional Recommendations developed with the primary aim to estimate Syringomyelia and Chiari Syndrome prevalence and incidence in North Western Italy, with special reference to symptomatic forms. METHODS: An agreement for the standardization of definitions, classifications, diagnostic criteria and surgical Recommendations was reached by the multidisciplinary Interregional Piemonte and Valle d'Aosta Chiari-Syringomyelia Consortium (Delphi method); next, in 2011 a census for Syringomyelia and Chiari Malformation was performed through the Interregional Piemonte and Valle d'Aosta Rare Disease Registry, integrated by a dedicated form in order to estimate prevalence and incidence. RESULTS: 436 patients, 292 females, met shared interregional diagnostic criteria. Syringomyelia prevalence was estimated in 4.84:100 000; Chiari Malformation prevalence was 7.74:100 000; incidence was 0.82:100 000 and 3.08:100 000 respectively. Demographics, neuroradiological parameters and aetiology were reported (in symptomatic and asymptomatic forms). Finally, symptoms and signs, familiar and natural history were analyzed. CONCLUSIONS: First Italian epidemiological data (prevalence, incidence) on Chiari and syringomyelia was collected, according to shared diagnostic Recommendations. Future perspectives include the adoption of these Recommendations at national level to standardize the access to diagnosis and care process and promote multicenter clinical trials.

Prevalence and Impact of Underlying Diagnosis and Comorbidities on Chiari 1 Malformation.

BACKGROUND: Chiari malformation type 1 affects approximately one in 1,000 people symptomatically, although one in 100 meet radiological criteria, making it a common neurological disorder. The diagnosis of underlying conditions has become more sophisticated, and new radiological markers of disease have been described. We sought to determine the prevalence and impact of additional comorbidities and underlying diagnoses in patients with Chiari malformation type 1 on symptomatology and surgical treatment. METHODS: A retrospective review of 612 pediatric patients with a Chiari malformation type 1 diagnosis and imaging data evaluated between 2008 and 2018 was performed. Because of extensive clinical heterogeneity, patients were separated into four categories based on their primary comorbidities (nonsyndromic, central nervous system, skeletal, and multiple congenital anomalies) to identify associations with age of onset, radiographic measurements, syringomyelia, and surgical treatment. RESULTS: The largest group had nonsyndromic Chiari malformation type 1 (70%) and the latest age at diagnosis of any group. In the syndromic group, 6% were diagnosed with a known genetic abnormality, with overgrowth syndromes being the most common. Patients with multiple congenital anomalies had the earliest Chiari malformation type 1 onset, the most severe tonsillar ectopia and obex position, and were overrepresented among surgical cases. Although there were no statistically significant differences between groups and rates of syrinx, we observed differences in individual diagnoses. CONCLUSION: The underlying diagnoses and presence of comorbidities in patients with Chiari malformation type 1 impacts rates of syringomyelia and surgery. Although most Chiari malformation type 1 cases are nonsyndromic, clinical evaluation of growth parameters, scoliosis, and joint hypermobility should be routine for all patients as they are useful to determine syringomyelia risk and may impact treatment.

Prevalence and clinical relevance of underlying pathological conditions in painful adolescent idiopathic scoliosis: a MRI-based study.

STUDY DESIGN: Cross-sectional comparative study. OBJECTIVES: Evaluate prevalence and clinical relevance of an underlying pathology in painful adolescent idiopathic scoliosis (AIS) patients after a non-diagnostic history, physical examination and spinal X-ray using Magnetic Resonance Image (MRI) as diagnostic tool. Discrepancies regarding indications of routine MRI screening in painful AIS patients are multifactorial. Few studies have investigated relationship and practical importance of painful AIS with an underlying pathology by MRI. METHOD: A total of 152-consecutive AIS patients complaining of back pain during a 36-month period were enrolled. All patients underwent whole-spine MRI after a non-diagnostic history, physical examination and spinal X-ray. Underlying pathologies were reported as neural and non-neural axis abnormalities based on MRI reports. Variables such as sex, age, constant or intermittent pain, night pain, back pain location (thoracic or lumbar pain), Cobb-angle and follow-up were evaluated as clinical markers to predict presence of underlying MRI pathologies. RESULTS: The presence of an underlying pathology was found by MRI in 54 painful AIS patients (35.5%). Isolated syringomyelia was the only neural axis abnormality found in 6 patients (3.9%). Non-neural axis abnormalities (31.6%) were composed by: 32 herniated nucleus pulposus, 5 vertebral disc desiccation, 4 ovarian cysts, 3 renal cysts, 2 sacral cysts, and 2 vertebral hemangiomas. There was no association with gender, age of presentation, initial coronal Cobb angle and follow up; with presence of an underlying pathology. Lumbar pain location was identified as an adequate clinical marker that correlated with presence of an underlying pathology (p = 0.01). CONCLUSIONS: Prevalence of underlying pathologies diagnosed by MRI in painful AIS was found high (35.5%), but it's clinical relevance and implication are debatable. The use of MRI did not affect orthopedic management of painful AIS patients who showed an underlying pathology. A thorough evaluation must be performed by clinicians; and discussed with patients and family prior to undergo further imaging management. LEVEL OF EVIDENCE: Level III.

The influence of concomitant syringomyelia on patient reported outcome following hind brain decompression.

Objectives: To evaluate the impact of concomitant syringomyelia and self-reported complications on patient reported outcome measures in patients undergoing hindbrain decompression for a Chiari 1 malformation.Methods: Prospective data collection of 95 patients who underwent Foramen magnum decompression between March 2011 and March 2015. Outcome evaluation was performed using the Core Outcome Measure Index questionnaire for neck (COMI-neck) and Gestalt impression (to assess improvement of headaches). Patients were split into two cohorts, those with and those without syringomyelia. Both cohorts were compared in all domains of the COMI neck questionnaires, headache, and complications. Non-parametric data were analysed with Wilcoxon signed rank, Mann-Whitney U and Fisher exact tests. Parametric data were analysed with Student T-test. SPSS Software was used for analysis.Results: 79 patients returned 1 year follow-up COMI-neck questionnaires. Thirty three had concomitant syringomyelia and 46 had no syringomyelia present. There was no statistically significant difference in patient reported outcomes (COMI-neck index median 4.5 +/- 3.3 vs 4.2+/-3.2; p = .376) between the syrinx and non-syrinx cohorts. However postoperative neck pain (median 4 +/- 3.35 vs 1 +/- 3.17; p 0.041) and arm/shoulder pain scores (2 +/- 3.38 vs. 0+/- 2.628; p 0.049) were significantly lower in the non-syrinx cohort. In both cohorts 57% patients had an improvement in headache. 92% patients were 'satisfied' with treatment and 63% stated that the operation 'helped'. 54% patients in the syrinx and 59% in the non-syrinx cohort self-reported complications. There was no statistical difference in outcomes of the patients with and without self-reported complications (p = .121).Conclusions: This study demonstrates that the clinical effectiveness of FMD is lower and reported complications are higher when evaluated by patient reported outcome measures as opposed to surgeon reported complications. Patients with and without concomitant syringomyelia showed equal overall outcomes, although neck and arm pain was worse in syrinx patients.

Opinion for different centers: surgical experience with Chiari malformation type I in children at Xuanwu Hospital, China.

PURPOSE: To evaluate the presenting symptoms, surgical strategy, and outcome in children with Chiari malformation type I (CM-I). METHODS: The medical records of children who were diagnosed with CM-I under 14 years old and subsequently underwent surgery for CM-I between 2014 and 2018 were reviewed. The medical records for presentation, radiological image, surgical intervention, and outcome were evaluated. RESULTS: Twelve children with CM-I and syringomyelia were included. All of the children were symptomatic. The most common presenting symptom was weakness, followed by scoliosis. All of them underwent posterior fossa decompression with/without duraplasty. Relief of preoperative pathologies and syringomyelia was experienced by all of them. CONCLUSIONS: The presenting symptoms of CM-I in children may be neurological deficits and scoliosis, which have a relationship with syringomyelia. Early recognition and a tailored operative procedure of CM-I in children could lead to good outcomes. Additional therapies for syringomyelia and scoliosis could be avoided.

Surgery for Chiari 1 malformation: the Lille experience.

PURPOSE: Decision-making in chronic tonsillar herniation (CTH) in children is complicated because many cases are diagnosed incidentally; on the other hand, its clinical impact may be underestimated. Furthermore, its surgical management is controversial. METHODS: In the present review, we tried to design a semi-quantitative approach to diagnosis, defining presenting symptoms as compatible, suggestive, or differential diagnoses. We expose our rationale for surgery. We review our experience with extensive posterior fossa decompression (PFD) with systematic dural opening and low threshold for tonsil resection. The aim is to achieve uncontroversial anatomical decompression. RESULTS: We operated 117 children during the last 10 years. Seventeen had complications, mostly hydraulic and minor; although most resolved without consequences, one patient died of unexplained cerebral vasospasm. At last control, 97% were clinically improved. No patient required reoperation for PFD. CONCLUSION: With proper patient selection, extensive PFD is a very efficient operation.

Results of the surgical treatment in children with Chiari malformation type I.

PURPOSE: Our study aimed to evaluate the quality of life of the patients operated due to Chiari malformation type 1 (CM-1) in the Department of Pediatric Neurosurgery, Medical University of Silesia in Katowice. METHODS: We performed a retrospective analysis of 11 patients diagnosed with CM-1 who were treated in our center in the years 2007 to 2016. There were 6 female and 5 male individuals. Short-term evaluation of the outcome was based on comparison of the presenting symptoms and radiological images before and after the surgical treatment. Long-term follow-up was carried out using survey questionnaires based on the Chicago Chiari Outcome Scale (CCOS) devised originally by Aliaga et al. RESULTS: Patients, based on their CCOS score were divided into three groups marked as "improved," "unchanged," and "worse," depending on a range of CCOS score: 13-16, 9-12, 4-8, respectively. The outcome of patients was as follows: 6 patients (55%) were evaluated as improved, and 5 (45%) as unchanged. No patient was classified as worse after surgery. Significant negative Spearman's correlation was found between the CCOS score and patients' age at the time of surgery (R = - 0.85, p = 0.0009). CONCLUSIONS: The decision of whether to operate pediatric patients with CM-1 should be considered very carefully. In our department, the main indication for surgery was the occurrence of clinical symptoms. Our study revealed that in the symptomatic patients, surgery improves their quality of life measured with CCOS.

Siringomielia no secundaria a Chiari. Actualización en fisiopatología y manejo / Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation

Introducción: Son muchos los conocimientos y publicaciones existentes sobre la siringomielia relacionada con la malformación de Chiari, pero existe poca difusión de este cuadro cuando no se presenta en relación con dicha malformación. Ello es importante ya que es una entidad propia que precisa de un conocimiento y manejo específico. Presentamos esta revisión con el objetivo de dar a conocer las ideas más aceptadas y difundidas a día de hoy al respecto de la fisiopatología, manejo y otros aspectos de la siringomielia no secundaria a malformación de Chiari. Desarrollo: Se ha realizado una revisión de la literatura más relevante en torno a esta patología, centrándose en su fisiopatología, presentación clínica, estudio diagnóstico y manejo. Conclusiones: La siringomielia no relacionada con malformación de Chiari es una entidad propia que precisa de un conocimiento adecuado en su profundidad para su sospecha, seguimiento y manejo adecuado. Ante el hallazgo de este cuadro debe realizarse un estudio detallado encaminado a intentar identificar la causa, quedando el tratamiento sintomático como opción de rescate

Introduction: Much has been published on syringomyelia related to Chiari malformation. In contrast, little is known about the condition when it is not associated with this malformation, but this presentation of syringomyelia constitutes a different entity and therefore requires specific management. We conducted a literature review to summarise the most accepted and widespread ideas about the pathophysiology, management and other aspects of syringomyelia unrelated to Chiari malformation. Development: We reviewed the most relevant literature on this condition, focusing on the pathophysiology, clinical presentation, diagnosis, and treatment. Conclusions: Syringomyelia unrelated to Chiari malformation is a distinct entity that must be well understood to guarantee correct diagnosis, monitoring, and management. When the disease is suspected, a thorough study should be conducted to identify its aetiology. Treatment must aim to eliminate the cause of the disease; symptomatic treatment should remain a second-line option

Management opinions from different centers (Rio de Janeiro).

INTRODUCTION: The surgical treatment of Chiari type 1 (CM1) malformation is controversial and depends largely on the preference of the surgeon. The evolution of neuroimaging resulted in an increased number of asymptomatic patients incidentally diagnosed. PURPOSE: To study retrospectively a population of 24 symptomatic patients with CM1 operated between 1999 and 2017 in which intraoperative ultrasonography (IOUS)-assisted posterior fossa-C1 decompression was used to decide whether the dura mater should be opened (CVD+) or not (CVD). RESULTS: Most of the patients complained of headache or neck pain, 15 had hydrosyringomyelia and 14 had some spinal cord involvement. Patients were categorized in improved, unchanged, or worse according the preoperative signs and symptoms. Overall, 19 patients improved, 3 deteriorated, and 2 remained unchanged. Among these, 4 out 5 had syringohydromyelia. CONCLUSIONS: IOUS-assisted posterior fossa-C1 decompression is our preferred option to treat CM1. Children submitted to intradural procedures, initially or subsequently, had increased postoperative complications. CSF fistula or pseudomeningocele was the major cause of complication. The final result seems to correlate with the preoperative neurological status.

Outcomes and complications for individual neurosurgeons for the treatment of Chiari I malformation at a children's hospital.

PURPOSE: The aim of this study was to evaluate the outcomes and complications for individual surgeons at British Columbia Children's Hospital for the treatment of Chiari I Malformation (CMI) in children. METHODS: This was a retrospective review of patients with CMI who had surgery from 1986 to 2015. We assessed the Chicago Chiari Outcome Scores (CCOS) and complication rates by surgeon. RESULTS: Seventy patients, 38 males and 32 females, underwent posterior fossa decompression including 14 extradural and 56 intradural approaches. Syringomyelia was present in 74.3%. Most syringomyelia improved with no difference between intradural and extradural surgeries. After initial surgery, 13 patients (18.6%) had complications including 2/14 (14.3%) of extradural and 11/56 (19.6%) of intradural surgeries. Two patients required surgical intervention for complications whereas 11 had transient complications. The complication rate by surgeon ranged from 11 to 20% for extradural (2 surgeons only) and 10.5 to 40% for intradural surgeries (4 surgeons). The CCOS ranged from 12 to 15 for extradural and 6 to 16 for intradural. The CCOS ranges for surgeons 1 and 2 were 12-15 and 13-15 respectively for extradural. The CCOS ranges for surgeons 1, 2, 3, and 4 were 12-16, 6-15, 12-16, and 12-16 respectively for intradural. Thirteen patients had a second surgery for CMI. The final CCOS was good in 86% and moderate in 14%. CONCLUSION: There was variability in surgeries performed at BCCH by different surgeons, with variations in CCOS and complication rates. This information is important during decision making, consent process, and for quality improvement.

"Management: opinions from different centers"-the Sankt Augustin experience.

OBJECTIVE: In this "how we do it" survey, we review our management regimen of symptomatic CM1 and provide an analysis of our institutional case series of "bony only" decompression of the craniocervical junction without dural opening. METHODS: In regard to the latter clinical symptomatology, neurological status, electrophysiology data, and pre- and post-surgical MRI were analyzed. Surgery was performed in standard fashion under IOM, evaluated by intraoperative ultrasound. RESULTS: We reviewed 22 patients (mean age at surgery 13 ± 7 years; 11 female, 11 male). Neck pain, occipital headaches, sensory symptoms, and dizziness were the predominating symptoms; 9% had central apnea, 5 patients had scoliosis, and 2 patients had a history of premature synostosis. On MRI, preoperative mean tonsillar herniation was 16.55 ± 6.19, compared to 14.25 ± 6.75 after surgery. About half of patients with syringomyelia (n = 11) experienced imagining improvement after surgery. Patients with neck pain, occipital headaches, dizziness, and sensory abnormalities benefited most from surgery. Of the 6 cases that presented with pathological SSEPs, 4 exhibited improved measurements after surgery. There were no postoperative complications. CONCLUSION: To conclude bony decompression for CM1 resulted in clinical and imaging wise improvement and can be viewed as a safe first-lane option for symptomatic CM1.