Search Results (53)

Background: Identifying the etiological factors of syringomyelia, which can cause progressive neurological deficits in the spinal cord, is critically important for both diagnosis and treatment. This study aimed to assess the cranial morphometric features of patients with idiopathic syringomyelia by conducting comparative analyses with individuals diagnosed with Chiari Type I, Chiari Type I accompanied by syringomyelia, and healthy controls, in order to elucidate the potential structural contributors to the pathogenesis of idiopathic syringomyelia. Methods: In this retrospective and comparative study, a total of 172 patients diagnosed with Chiari Type I and/or syringomyelia between 2016 and 2024, along with 156 radiologically normal individuals, were included. The participants were categorized into four groups: healthy controls, Chiari Type I, Chiari Type I with syringomyelia, and idiopathic syringomyelia (defined as syringomyelia without an identifiable cause). Midline sagittal T1-weighted MR images were used to obtain quantitative measurements of the posterior fossa, cerebellum, intracranial area, and foramen magnum. All measurements were stratified and statistically analyzed by sex. Results: In cases with idiopathic syringomyelia, both the posterior fossa area and the cerebellum/posterior fossa ratio differed significantly from those of healthy controls. In male patients, the foramen magnum diameter was significantly larger in the Chiari + syringomyelia group compared with the idiopathic group. A significant correlation was found between the degree of tonsillar descent and selected morphometric parameters in female subjects, whereas no such correlation was observed in males. Both Chiari groups exhibited significantly smaller posterior fossa dimensions compared with the healthy and idiopathic groups, indicating greater neural crowding. Additionally, in Chiari Type I patients, increasing degrees of tonsillar descent were associated with a decreased incidence of syringomyelia. Conclusions: Anatomical variations such as a reduced posterior fossa area or altered foramen magnum diameter may contribute to the pathogenesis of idiopathic syringomyelia. Cranial morphometric analysis appears to offer diagnostic value in these cases. Further prospective, multicenter studies incorporating advanced neuroimaging modalities, particularly those assessing cerebrospinal fluid dynamics, are warranted to better understand the mechanisms underlying syringomyelia of unknown etiology. Full article

Background: Chiari-like malformation (CM) and syringomyelia (SM) are commonly observed conditions in Pomeranian dogs. Affected dogs may develop clinical signs that significantly impact quality of life. Therefore, it is crucial to select only unaffected dogs for breeding. However, the progression of CM/SM has not been fully elucidated. Dogs that are unaffected or mildly affected may progress to severe SM over time. The primary aim of this study is to investigate the progression of CM/SM through repeated MRI scans. A secondary objective is to evaluate the effect of furosemide treatment on syrinx sizes, given its frequent prescription. Methods: Pomeranians that underwent two CM/SM screenings between 2015 and 2025 were included. CM/SM classifications were assessed, and quantitative syrinx measurements were conducted. Maximum syrinx diameter (MSD) and maximum syrinx-to-spinal cord diameter ratio (MSD/SCD-r) were measured and documented. Dogs were classified based on the progression of SM. Furosemide treatment was documented, and its effect on syrinx size was compared with that in dogs not receiving furosemide. Results: At the time of the second MRI, 39.6% of dogs either developed SM or showed substantial progression, whereas 12.5% demonstrated partial recovery. Of the dogs initially classified as free from SM, 20.7% had developed the condition. A significant increase was observed in both MSD (p = 0.0058) and MSD/SCD-r (p = 0.0038) between MRI1 and MRI2. Notably, the change in MSD between MRI1 and MRI2 was statistically significantly smaller in dogs treated with furosemide compared to untreated dogs (p = 0.030). Conclusions: These findings indicate that syrinx dimensions are dynamic and may fluctuate over time, although a general trend toward progression is observed. Furthermore, furosemide may mitigate the progression of SM. Full article

Background: Patients with Ehlers–Danlos Syndrome (EDS) and craniocervical instability are treated with extensive craniocervical fixation. A new argument and treatment are proposed related to filum terminale collagen dysfunction: the Neuro-Cranio-vertebral syndrome theory (NCVS). Objectives: To analyse clinical manifestation and imaging features of NCVS patients associated with EDS compared with 373 NCVS-affected controls, to propose an aetiopathogenic mechanism for NCVS in EDS patients, and to analyse and assess postoperative changes in NCVS patients with EDS after sectioning of the filum terminale. Methods: We conducted a retrospective study and selected ten patients diagnosed with EDS and NCVS. We present the images, signs, and symptoms in these cases, compared to those of 373 patients with NCVS alone. In addition, we report postsurgical findings in four EDS–NCVS patients after sectioning of the filum terminale. Results: Patients with EDS and NCVS had more cranial and vertebral symptoms. There were also significant differences in the neurological signs present in EDS–NCVS compared to those in NCVS alone. Patients who underwent sectioning of the filum terminale showed a significant improvement in signs and symptoms. Conclusions: The concept of craniocervical instability due to EDS does not explain a large number of neurological signs and symptoms, which seem to fit better in our new NCVS theory. Surgical treatment would only involve sectioning the filum terminale, while cervical fusion would never be justified in such patients. Full article

Background: Syringomyelia is a complex neurological disorder characterized by a fluid-filled cavity (syrinx) within the spinal cord, frequently resulting from altered cerebrospinal fluid (CSF) dynamics. While its clinical manifestations are diverse, orthopedic complications such as scoliosis, pes cavus, and Charcot arthropathy may represent early diagnostic clues yet are often under-recognized. Methods: This comprehensive review synthesizes the current literature on the pathophysiology, clinical presentation, diagnostic strategies, and management approaches of syringomyelia, with a specific emphasis on its orthopedic manifestations. Additionally, we present a detailed case of neuropathic shoulder arthropathy associated with advanced syringomyelia. Results: Orthopedic involvement in syringomyelia includes progressive spinal deformities and neurogenic joint destruction, particularly affecting the shoulder and elbow. Scoliosis is frequently observed, especially in association with Chiari malformations, and may precede neurologic diagnosis. Charcot joints result from impaired proprioception and protective sensation. The case presented illustrates the diagnostic challenges and therapeutic dilemmas in managing advanced neuro-orthopedic complications in syringomyelia. Conclusions: Syringomyelia should be considered in the differential diagnosis of atypical musculoskeletal presentations. Early recognition and multidisciplinary management are essential to prevent irreversible orthopedic sequelae. Conservative treatment remains the mainstay in stable cases, while surgery is reserved for progressive disease. Orthopedic assessment plays a pivotal role in the diagnostic pathway and long-term care of affected patients. Full article

Introduction: The presence of syringomyelia associated with Dandy-Walker malformation is rarely described in adults. Case report: We report a case of a 28-year-old woman with a history of Dandy-Walker malformation who developed syringomyelia. She had been previously treated in childhood with a ventriculoperitoneal and cystoperitoneal shunt for hydrocephalus, but over time she developed progressive neurological symptoms, including numbness and weakness in the upper extremities. Magnetic resonance imaging revealed a syrinx extending from C4 to T1 associated with large posterior fossa cyst. The patient was treated with cyst fenestration and cystoperitoneal shunts were removed, with complete resolution of symptoms and disappearance of syrinx. Discussion: A literature review revealed only 6 cases of syringomyelia associated with Dandy-Walker malformation in adults. The pathophysiology of this entity is multifactorial and may be related to obstructed cerebrospinal fluid flow, altered pressure dynamics, and formation of arachnoid adhesions. Conclusions: Individualized surgical approaches are essential for optimizing outcomes in this rare condition. Further research is needed to standardize treatment protocols and clarify underlying mechanisms and help to improve the management of these patients. Full article

Syringomyelia detected in both animals and humans may cause a variable degree of discomfort and its etiology is commonly unidentified. The aim of this study was to compare the outcome in dogs with those having syringomyelia of different etiology. Dogs with syringomyelia were subdivided into two groups: syringomyelia associated with Chiari-like malformation (S-CLM) (15 dogs) and syringomyelia of other etiology (SOA) (15 dogs). Age onset of S-CLM clinical signs was earlier compared to SOA (mean S-CLM and SOA values: 50.53 and 97.6 months, respectively, p = 0.021). Two neurological dysfunction scoring systems alongside nociception values were lower in SOA compared to S-CLM (mean values for neurological dysfunction scoring system SOA and S-CLM: 5.87 and 4.2, respectively, p = 0.032) (mean values for nociception SOA and S-CLM: 20.97 and 10.03, respectively, p = 0.03). Symptomatic therapy included combinations of corticosteroids, gabapentin (10/15, 66.6%) in S-CLM and NSAID +/− gabapentin (8/15, 53.3% and 9/15, 60%, respectively) in SOA dogs. Eight S-CLM dogs (53.4%) improved with symptomatic therapy and eleven were still alive; however, most SOA dogs (9/15, 73.4%) died/were euthanized by the end of this study. SOA dogs demonstrated more severe neurological signs compared to S-CLM, although the outcome between the two groups was not associated (p = 0.211). Full article

Background: Chiari-like malformation (CM) and syringomyelia (SM) are commonly observed disorders in the Pomeranians. Both disorders can lead to pain and diminish quality of life to varying extents. In veterinary medicine, affected dogs are often treated with gabapentin or pregabalin, which contrasts with human medicine, where the tricyclic antidepressants amitriptyline and nortriptyline are the first-choice treatments. Methods: affected dogs were treated with either amitriptyline or gabapentin. If a pre-syrinx was present, furosemide was also added. Owners were asked to score the clinical signs they observed (ORCS). Results: 90 Pomeranians were included, of which 62 received amitriptyline and 28 received gabapentin. Adding furosemide did not have an influence on the outcome. A logistic regression analysis with the number of ORCS, duration of ORCS, treatment (amitriptyline or gabapentin), and outcome (responder or non-responder) as the dependent variable revealed that the number of ORCS had an Odds of 1123 (p = 0.03). Duration of the ORCS (Odds 1.027; p = 0.45) and treatment group had a low influence (Odds 1.258; p = 0.65). Conclusions: In this study, amitriptyline demonstrated a moderate, though not statistically significant, more favorable effect on CM/SM pain compared to gabapentin. Adding furosemide to either of these two treatments did not influence the outcome. The outcome for dogs affected by CM/SM is not influenced by MRI grading; however, the number of ORCS and a longer duration negatively impact the outcome. A dose of 1 to 2 mg/kg body weight of amitriptyline administered twice daily proved to be more effective in several dogs. Full article

(1) Background: Surgery for Chiari I malformation (CMI) is indicated when typical clinic-radiological features (syringomyelia, exertional headaches, sleep apnea syndrome, and tetraparesis) are present. Sometimes, patients have atypical complaints suggestive of otolaryngological (ENT) involvement, and it is sometimes difficult for the neurosurgeon to determine if these complaints are related to the CMI. Our aim was to describe postural control patterns in children with CMI using computerized dynamic posturography. To our knowledge, this is the first study addressing postural instability in pediatric CMI patients. (2) Methods: Twenty-eight children aged 6 to 17 years with both radiologically confirmed CMI and clinical ENT complaints were included. The children were separated into two groups, operated and non-operated patients, based on neurosurgical indication. Epidemiologic and posturographic results (CDP—Equitest^®) were compared between both groups, as well as pre- and postoperatively in Group 2. (3) Results: In Group 2 patients, significant improvement of global SOT was found after intervention. When the three sensorial aspects of postural control calculated by the system were independently assessed, the greatest improvement was in the vestibular ratio. We also observed an altered CoG pattern (“lateral deviation”) in the Group 2 patients, which significantly differed from those in Group 1. Lateral deviation was significantly reduced postoperatively in the Group 2 patients. A correspondence between preoperative MRI and the side of lateralization on posturography was found in four children, but this cannot be regarded as significant due to the low number of patients. (4) Conclusions: Postural control seems to improve after surgery for CMI in children, mostly due to the improvement in vestibular function. There is a correspondence between the side of lateral deviation and the side of greatest tonsillar descent on MRI and perioperatively. Further studies are needed to support these results and to confirm the utility of CDP in CMI patients. Full article

Background: Chiari malformation type 1 (CM1) remains a complex neurosurgical condition with ongoing debate regarding its optimal management. Methods: This narrative review examines key controversies surrounding the pathophysiology, surgical indications, and treatment strategies for CM1. Results: We highlight the challenges posed by the wide spectrum of CM variants and the evolving understanding of its association with syringomyelia, basilar invagination, and craniovertebral instability. Emerging surgical techniques, including minimally invasive approaches and the use of new technologies such as endoscopes and exoscopes, are evaluated for their potential to improve outcomes. Recent consensus guidelines are also discussed. Conclusions: The need for individualized treatment plans for CM1 is emphasized, with special focus put on the connection between novel pathophysiological insights, technological advancements and opportunities for a more nuanced surgical management. Further research is necessary to establish solid foundations for more individualized treatments. Full article

Background: Chiari-like malformation (CM), Syringomyelia (SM) and middle ear effusion (MEE) are frequently observed disorders in Cavalier King Charles Spaniels (CKCSs), Pomeranians, and less frequently the Griffon. There are a few reports in which small dogs have been identified as suffering from CM/SM. Methods: From all MRI centers based in the Netherlands, MRI scans for small dog breeds performed for various reasons over a period of 8 years were collected. Scans that enabled a CM and/or SM evaluation were included and evaluated. Results: In total, 177 MRI scans were included. CM could be evaluated in 163 out of 177 dogs. In 63 dogs, no CM was observed, while 100 dogs had CM. SM could be evaluated in 144 out of 177 dogs. No SM was seen in 56 dogs and SM was seen in 88 dogs. Both CM and SM were seen in high frequency in the Chihuahua, French Bulldog, Griffon, and Pug but did also occur in lower frequencies in various other small breed dogs and crosses. CM and/or SM does occur in various other small dog breeds and crosses suggesting that it is indeed a type-related disorders. As it can cause serious clinical signs breeders should be aware of the risk of CM/SM when breeding with these small dog breeds. Full article

This review highlights the need for therapeutic guidelines for syringomyelia associated with tethered cord syndrome (TCS) caused by spinal dysraphism (SD). A comprehensive literature review was conducted, selecting twelve articles to analyze common therapeutic strategies. Surgical cord untethering alone has recently become a preferred treatment, with 45 ± 21.1% of patients experiencing remission or improvement, 47 ± 20.4% unchanged and asymptomatic, and 4 ± 8% worsened. Untethering with direct surgical drainage for the syrinx had better outcomes than untethering alone (78% vs. 45%, p = 0.05). Terminal syringostomy was beneficial for syrinxes extending to the filum terminale but not for asymptomatic small syrinxes with a syrinx index < 0.4. Syrinx shunting was recommended for symptomatic large syrinxes (>2 cm in length and syrinx index > 0.5). Various shunt procedures for syrinxes are still advocated, mainly for refractory syringomyelia in Chiari malformation, posttraumatic cases, SD, or other causes. Personalized surgical methods that address the root cause of syringomyelia, particularly those improving cerebrospinal fluid flow, offer promising results with minimized complications. Ongoing studies are required to enhance management strategies for syringomyelia associated with TCS, optimize patient outcomes, and reduce the risk of recurrent symptoms. Full article

D-waves (also called direct waves) result from the direct activation of fast-conducting, thickly myelinated corticospinal tract (CST) fibres after a single electrical stimulus. During intraoperative neurophysiological monitoring, D-waves are used to assess the long-term motor outcomes of patients undergoing surgery for intramedullary spinal cord tumours, selected cases of intradural extramedullary tumours and surgery for syringomyelia. In the present manuscript, we discuss D-wave monitoring and its role as a tool for monitoring the CST during spinal cord surgery. We describe the neurophysiological background and provide some recommendations for recording and stimulation, as well as possible future perspectives. Further, we introduce the concept of anti D-wave and present an illustrative case with successful recordings. Full article

Background/Objectives: The management of Chiari malformations (CMs) remains a clinical challenge and a topic of great controversy. Results may vary between children and adults. The purpose of the current single-center study is to critically assess the one-year surgical outcomes of a cohort of 110 children with CM-1 or CM-1.5 who were treated using “posterior fossa reconstruction” (PFR), a surgical technique described in 1994 that has since been used in both adults and children. We also review the literature and discuss the possible causes of the drawbacks and pitfalls in children in whom PFR was ineffective in controlling the disease. Methods: The present cohort was selected from a prospective registry of adults and children with CMs collected since 2006. Patients included in this study were selected from a group of children with CMs who were operated on in our Pediatric Neurosurgical Unit between 1 January 2007 and 31 November 2023. Surgical outcome was defined based on clinical and neuroradiological results as very good, good, or bad. Results: The mean age of our child cohort was 9.9 ± 4.7 years, with 54 girls (49%) and 56 boys (51%). Sixty-six children had CM-1 (60%) while forty-four had CM-1.5 (40%). Following surgery, there was no neurological worsening or death among the children. Most children (70%) had an uneventful recovery and were discharged home on average one week after surgery. However, in 33 children (30%), we recorded at least one postoperative adverse event. Aseptic meningitis syndrome was the most frequent adverse event (n = 25, 22.7%). The final surgical outcome was evaluated one year after PFR by using both clinical and neuroradiological results. The one-year surgical outcome was excellent in 101 children (91.9%), good in 5 (4.5%), and bad in 4 (3.6%). Conclusions: PFR significantly enlarges the volume of the posterior fossa and recreates a CSF environment that generates buoyancy of the cerebellum, with a high percentage of excellent and good clinical results evaluated one year post-surgery. Full article

Background: The aim of Part II of this two-part study is to describe and analyze the association of various aspects and measurements related to the morphometry of the skull and craniocervical region to CM/SM status of Pomeranians, by means of computed tomography (CT) and magnetic resonance imaging (MRI). Methods: Prospectively, Pomeranians were included that underwent both CT and MRI studies of the head and cervicothoracic vertebral column. For those cases where qualitative classifications differed between observers, the experienced observer re-evaluated the studies and decided on a final classification that was used for further analysis. For quantitative measurements, the means of the observers’ measurements were used for analysis. Results: Among statistically significant differences in measurements, we found that dogs with SM had a significantly shorter clivus length based on both MRI (p = 0.01) and CT measurements (p = 0.01), and a significantly smaller caudal cranial fossa area based on both MRI (p = 0.02) and CT measurements (p = 0.02). Conclusions: Significant morphometrical differences were identified between dogs with or without CM/SM. The findings in this study add to those already described in other breeds and provide further insight into factors that may play a role in the pathogenesis of CM/SM in Pomeranians. Full article

Background: Currently, there are no diagnostic imaging-based studies that have focused specifically on the craniocervical morphology of Pomeranian dogs in relation to Chiari-like malformation and syringomyelia (CM/SM). The aims of Part I of this two-part study are to assess the intra-observer, interobserver, and intermodality reliability and agreement for various aspects of the craniocervical morphology of Pomeranians with and without CM/SM. Methods: Prospectively, Pomeranians were included that underwent both CT and MRI studies of the head and cervicothoracic vertebral column. Two observers (experienced and novice) independently performed CM and SM classifications, qualitative assessments, and quantitative measurements. The experienced observer performed these assessments twice. A third observer (experienced) performed CM and SM normal or abnormal classifications. Results: Ninety-nine (99) dogs were included. Interobserver reliability was influenced by observer experience level. For the experienced versus novice observers, substantial interobserver agreement was found for classification of SM as normal or abnormal (Cohen’s kappa = 0.63), while interobserver agreement was fair for classification of SM as normal or abnormal (Cohen’s kappa = 0.31). Interobserver, intra-observer, and intermodality reliability were variable for different measurements and assessments, and best overall for the CT-based measurements. Conclusions: Interobserver reliability and agreement results should be taken into account in the evaluation of results of future studies as well as the evaluation of imaging studies of Pomeranians presented clinically. Full article