RESUMEN
BACKGROUND: Central neuropathic pain after foramen magnum decompression (FMD) for Chiari malformation type 1 (CM-1) with syringomyelia can be residual and refractory. Here we present a case of refractory central neuropathic pain after FMD in a CM-1 patient with syringomyelia who achieved improvements in pain following spinal cord stimulation (SCS) using fast-acting sub-perception therapy (FAST™). CASE PRESENTATION: A 76-year-old woman presented with a history of several years of bilateral upper extremity and chest-back pain. CM-1 and syringomyelia were diagnosed. The pain proved drug resistant, so FMD was performed for pain relief. After FMD, magnetic resonance imaging showed shrinkage of the syrinx. Pain was relieved, but bilateral finger, upper arm and thoracic back pain flared-up 10 months later. Due to pharmacotherapy resistance, SCS was planned for the purpose of improving pain. A percutaneous trial of SCS showed no improvement of pain with conventional SCS alone or in combination with Contour™, but the combination of FAST™ and Contour™ did improve pain. Three years after FMD, percutaneous leads and an implantable pulse generator were implanted. The program was set to FAST™ and Contour™. After implantation, pain as assessed using the McGill Pain Questionnaire and visual analog scale was relieved even after reducing dosages of analgesic. No adverse events were encountered. CONCLUSION: Percutaneously implanted SCS using FAST™ may be effective for refractory pain after FMD for CM-1 with syringomyelia.
Asunto(s)
Malformación de Arnold-Chiari , Neuralgia , Estimulación de la Médula Espinal , Siringomielia , Humanos , Siringomielia/complicaciones , Femenino , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/cirugía , Anciano , Neuralgia/etiología , Neuralgia/terapia , Estimulación de la Médula Espinal/métodos , Dolor Postoperatorio/terapia , Dolor Postoperatorio/etiología , Resultado del TratamientoRESUMEN
Background and purpose:
Syringomyelia is a neurological condition in which a longitudinal fluid-filled cavity is formed within the spinal cord. It usually occurs in the cervical region and is associated with Chiari malformation, infections, trauma, and tumors of the spinal cord. However, syringomyelia associated with cervical disc disease (SCD) is very rare and only a few cases have been reported so far. This case report presents the clinical and radiological findings of 13 cases of SCD to describe the properties of SCD and explore the nature of the relationship between syringomyelia and cervical disc disease.
. Methods:SCD was diagnosed in 13 using MRI findings, including coexistence of syringomyelia and cervical disc disease, presence of narrowed cervical subarachnoid space secondary to the cervical disc herniation or cervical local kyphosis associated with cervical disc degeneration or herniation, and the cervical disc herniation or segmental kyphosis and syrinx should be located within the same levels. The MRI findings were used to grade the syrinx and determine whether the cervical disc herniation or local kyphosis was located at the proximal or distal end of the syrinx.
. Results:All patients had single-level disc herniation or kyphosis, the most common level being C5–6 (n = 6), followed by C6–7 (n = 4) and C4–5 (n = 3). Eight patients had a distal type (disc disease located in the proximal end of the syrinx) SCD while five had the proximal variety (cervical disc disease located in the distal end of the syrinx). The average length of the syrinx was two vertebral segments. Surgery was performed in five cases and some degree of syrinx resolution was observed in all of them.
Discussion – The main cause of syringomyelia is obstruction of cerebrospinal fluid (CSF) pathways; total obstruction could cause distal syrinx, whereas partial obstruction could cause proximal or distal syrinxes. Restoration of CSF pathways may result in some degree of resolution of syringomyelia. A causal association may exist between cervical disc disease and cervical syringomyelia but needs further exploration.
SCD is a mild form of syringomyelia with symptoms primarily arising due to disc herniation or local kyphosis. The surgical treatment of the cervical disc disease is sufficient and results in a syringomyelia resolution of some degree.
.Asunto(s)
Vértebras Cervicales , Degeneración del Disco Intervertebral , Desplazamiento del Disco Intervertebral , Imagen por Resonancia Magnética , Siringomielia , Humanos , Siringomielia/diagnóstico por imagen , Siringomielia/complicaciones , Vértebras Cervicales/diagnóstico por imagen , Vértebras Cervicales/patología , Masculino , Femenino , Persona de Mediana Edad , Adulto , Desplazamiento del Disco Intervertebral/complicaciones , Desplazamiento del Disco Intervertebral/diagnóstico por imagen , Degeneración del Disco Intervertebral/diagnóstico por imagen , Degeneración del Disco Intervertebral/complicaciones , Anciano , Cifosis/diagnóstico por imagen , Cifosis/complicaciones , Cifosis/etiologíaRESUMEN
ABSTRACT: Currarino syndrome (CS) is a rare congenital syndrome characterized by a triad of anorectal malformation, sacral deformity, and presacral mass. In about 50% of cases, it is caused by HLXB9 gene mutation in chromosome 7q36. A 13-month-male child presented with presacral discharging sinus with a history of surgery for anorectal malformation and perineal fistula at the time of birth. On detailed investigation, the child revealed to have anal atresia, hemisacrum, and presacral mass. Histopathology of presacral mass showed features of immature teratoma. The presacral mass in CS is mostly an anterior myelomeningocele or presacral teratoma. The development of immature teratoma in presacral mass is very rare. The histopathological identification of immature component of teratoma in the presacral mass of CS is important for risk stratification and further management. Suspicion of CS should be raised in any child presenting with partial phenotype of the triad.
Asunto(s)
Canal Anal , Anomalías del Sistema Digestivo , Recto , Sacro , Siringomielia , Teratoma , Humanos , Teratoma/patología , Teratoma/cirugía , Teratoma/diagnóstico , Masculino , Canal Anal/anomalías , Canal Anal/cirugía , Canal Anal/patología , Sacro/anomalías , Sacro/cirugía , Sacro/patología , Anomalías del Sistema Digestivo/cirugía , Anomalías del Sistema Digestivo/diagnóstico , Anomalías del Sistema Digestivo/patología , Anomalías del Sistema Digestivo/genética , Siringomielia/cirugía , Siringomielia/genética , Siringomielia/patología , Siringomielia/diagnóstico , Siringomielia/diagnóstico por imagen , Lactante , Recto/anomalías , Recto/cirugía , Recto/patología , Ano Imperforado/cirugía , Ano Imperforado/diagnóstico , Ano Imperforado/genética , Ano Imperforado/patologíaRESUMEN
BACKGROUND AND IMPORTANCE: Syringomyelia, or the formation of fluid-filled cysts within the spinal cord, associated with delayed spinal arachnoiditis is an uncommon complication of aneurysmal subarachnoid haemorrhage. To date, about 18 cases have been reported in medical literature, with just two reported in patients under the age of 35 years. CLINICAL PRESENTATION: A 27-year-old female patient complained of sudden, severe headaches in the occipital region, nuchal rigidity, and drowsiness when she presented at our institution. A head computed tomography scan revealed intraventricular bleeding in the lateral and fourth ventricles with more extensive haemorrhaging in the frontal horns. A left posterior inferior cerebellar artery (PICA) aneurysm was confirmed via digital subtraction angiogram, and endovascular embolization was done. Two years later, the patient reported intense pain in the lower back along with symptoms suggestive of spinal cord compression. Spinal magnetic resonance imaging (MRI) showed spinal adhesions from C1 to L4, syringomyelia with some vasogenic oedema extending from T3 to T9 level, and a cyst in the lumbar region. Consequently, a right hemilaminectomy was performed along with microsurgical release of arachnoid adhesions and placement of a subdural drain. Radiological and symptomatic improvements were observed. Since then, the patient's clinical condition has remained stable during the past three years of follow-up visits. CONCLUSIONS: Literature on optimal treatment modalities and patient prognosis is scarce and debated. The time for symptom improvement depends on the level and extent of spinal cord involvement. Rehabilitation may be required for most patients, as complete symptomatic recovery may not be attainable.
Asunto(s)
Aracnoiditis , Hemorragia Subaracnoidea , Siringomielia , Humanos , Femenino , Aracnoiditis/complicaciones , Aracnoiditis/etiología , Adulto , Siringomielia/etiología , Siringomielia/diagnóstico por imagen , Siringomielia/complicaciones , Siringomielia/cirugía , Hemorragia Subaracnoidea/complicaciones , Hemorragia Subaracnoidea/cirugía , Hemorragia Subaracnoidea/etiologíaRESUMEN
INTRODUCTION: A subset of children with Chiari 1 malformation (CM-1) have a 4th ventricle arachnoid veil-a thin membrane covering the outlet of the 4th ventricle. Studies suggest that failure to disrupt this veil during posterior fossa decompression can reduce the likelihood of syringomyelia resolution. However, there is no reliable method for predicting the presence of the veil without direct surgical exploration. This study aims to evaluate the association between pre-operative symptoms, radiographic measurements, and the arachnoid veil. METHODS: A retrospective review of an institutional database of children evaluated for CM-I was conducted. For patients treated with surgery, operative notes were reviewed to determine if an arachnoid veil was present. Logistic regression was used to test for relationship of clinical variables and radiographic measurements with the presence of an arachnoid veil. RESULTS: Out of 997 children with CM-1, 226 surgical patients were included in the analysis after excluding those with inadequate documentation. An arachnoid veil was found in 23 patients (10.2%). Larger syrinx, spinal canal, and thecal sac diameters were significantly associated with the presence of a veil, with odds ratios of 1.23 (95% CI 1.2-1.48; p = 0.03), 1.27 (95% CI 1.02-1.59; p = 0.03), and 1.35 (95% CI 1.03-1.77; p = 0.03), respectively. No significant associations were found with any signs or symptoms. CONCLUSIONS: Arachnoid veil was present in 10% of cases. Radiographic measurements indicating larger syrinx size were the only variables found to be significantly associated with an arachnoid veil. Exploration of the 4th ventricular outlet is recommended for CM-I decompression in the setting of expansile syringomyelia.
Asunto(s)
Malformación de Arnold-Chiari , Cuarto Ventrículo , Humanos , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/diagnóstico por imagen , Femenino , Masculino , Estudios Retrospectivos , Niño , Preescolar , Adolescente , Cuarto Ventrículo/diagnóstico por imagen , Cuarto Ventrículo/cirugía , Siringomielia/cirugía , Siringomielia/diagnóstico por imagen , Siringomielia/complicaciones , Lactante , Descompresión Quirúrgica/métodos , Aracnoides/cirugía , Aracnoides/diagnóstico por imagenRESUMEN
PURPOSE: Chiari I malformation (CM-I) in pediatric patients can impose substantial neurologic and functional impairment. Additionally, the presence of syrinx is often a harbinger of clinical compromise, but little attention has been devoted to identifying features associated with syrinx development and the clinical impact of syrinx resolution. Therefore, this study aims to identify clinical and radiographic variables associated with preoperative syrinx presence and postoperative syrinx reduction in pediatric patients with CM-I and determine the relationship between postoperative syrinx reduction and clinical symptom improvement. METHODS: The authors performed a retrospective analysis of 435 consecutive pediatric patients who underwent surgical treatment of CM-I from 2001 to 2021 at a single tertiary pediatric medical center. All patients underwent pre- and postoperative MRI, and clinical and radiographic variables were recorded and subject to inferential analysis. RESULTS: Syrinx at presentation was independently associated with symptoms of spinal cord dysfunction at presentation (OR 2.17 (95% CI 1.05-4.48); p = 0.036), scoliosis (OR 5.33 (2.34-10.86); p = 0.001), and greater pB-C2 (posterior basion to C2 distance) measurement length (OR 1.14 (95% CI 1.01-1.30); p = 0.040). Syrinx at presentation was inversely associated with tussive headaches at presentation (OR 0.27 (95% CI 0.16-0.47); p = 0.001) and cranial nerve deficits at presentation (OR 0.49 (95% CI 0.26-0.92); p = 0.025). Postoperatively, patients with radiographic evidence of syrinx improvement had greater rates of symptom improvement (93.1% vs 82.1%; p = 0.049), better CCOS scores (15.4 vs 14.2; p = 0.001), and decreased rates of readmission (6.0% vs 25.0%, p = 0.002) and reoperation (0.5% vs 35.7%; p = 0.001). The difference in syrinx resolution was similar but not statistically significant (10.3% vs 16.7%; p = 0.251). AO joint anomaly (OR 0.20, 95% CI 0.04-0.95; p = 0.026) and foramen magnum diameter (OR 1.12, 95% CI 1.00-1.25; p = 0.049) were the only independent predictors of syrinx improvement, and surgical technique was the only predictor for syrinx resolution (OR 2.44, 95% CI 1.08-5.50; p = 0.031). Patients that underwent tonsil reduction surgery whose syrinx improved had a wider foramen magnum diameter than those whose did not improve (34.3 vs 31.7; p = 0.028). CONCLUSIONS: Radiographic syrinx improvement is associated with greater rates of symptom improvement and less readmissions and reoperations for CM-I. AO joint anomalies and narrower foramen magnums were independent risk factors for the lack of syrinx improvement. These novel insights will help guide preoperative patient counseling, pre- and intraoperative surgical decision-making, and postoperative clinical prognostication in the treatment of pediatric CM-I.
Asunto(s)
Malformación de Arnold-Chiari , Siringomielia , Humanos , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/diagnóstico por imagen , Malformación de Arnold-Chiari/complicaciones , Masculino , Femenino , Siringomielia/cirugía , Siringomielia/diagnóstico por imagen , Niño , Estudios Retrospectivos , Adolescente , Preescolar , Resultado del Tratamiento , Imagen por Resonancia Magnética , LactanteRESUMEN
Morvan's syndrome is a rare, complex autoimmune syndrome comprising peripheral nerve hyperexcitability, dysautonomia, insomnia, and encephalopathy. In this case report, we highlight the clinical and pathological findings of an elderly gentleman who presented to us with clinical features of Morvan's syndrome associated with anti-contactin-associated protein 2 (CASPR-2) antibodies. Histopathology [Figure 3] revealed cortical atrophy with gliosis and mild microglial proliferation. Microglial activation and gliosis were observed in the hippocampus, hypothalamus, and thalamus. Brainstem showed multifocal inflammation. Mild inflammation was observed in the leptomeninges. Morvan's syndrome is an autoimmune disease with antibodies targeted against CASPR within the voltage-gated potassium channel (VGKC) complex. Early diagnosis and treatment play a key role in the management of patients. Most patients show a good response when treated with plasmapheresis and steroids. This patient presented to us late into the illness and succumbed.
Asunto(s)
Autopsia , Humanos , Masculino , Proteínas del Tejido Nervioso/metabolismo , Anciano , Siringomielia/patología , Autoanticuerpos , Encéfalo/patología , Encéfalo/diagnóstico por imagen , Proteínas de la MembranaRESUMEN
The conformation of spinal arachnoid diverticula (SAD) and their clinical implications are poorly characterized in dogs. This retrospective cross-sectional study describes different SAD conformations in dogs and aims to identify if there is an association between SAD conformation and clinical features, localization, syringomyelia (SM) presence, concurrent vertebral condition, treatment option, and short as well as long-term outcome. Sixty-two dogs were included (12 cervical and 50 thoracolumbar SAD). All dogs with a cervical SAD had a cranial tethered conformation and were not included in the statistical analysis. Half of the dogs with a thoracolumbar SAD were cranial tethered, and the other half were caudal tethered. SM associated with SAD had a moderate prevalence in the cervical region (58.3%) and a high prevalence in the thoracolumbar region (82%). All dogs with the presence of SM and caudal tethered SAD had a cranial positioned SM, and all dogs with SM and a cranial tethered SAD had a caudal positioned SM. The SM absolute length and SM length/L2 ratio were significantly higher (P = .018, respectively) in the caudal tethered SAD compared with the cranial tethered SAD. The short-term outcome was statistically different (P = .045) between caudal and cranial tethered thoracolumbar SAD, but not the long-term outcome (P = .062). Multivariable logistic regression identified thoracolumbar caudal tethered SAD conformation had a better short-term outcome (P = 0.017, OR: 0.043, CI: 0.003-0.563), independently of SM length measurements. SAD conformation in dogs can influence SM formation. A possible link between short-term outcome and SAD conformation was found, but further research is warranted.
Asunto(s)
Enfermedades de los Perros , Perros , Animales , Enfermedades de los Perros/diagnóstico por imagen , Enfermedades de los Perros/patología , Estudios Retrospectivos , Estudios Transversales , Femenino , Masculino , Quistes Aracnoideos/veterinaria , Quistes Aracnoideos/diagnóstico por imagen , Siringomielia/veterinaria , Siringomielia/diagnóstico por imagen , Divertículo/veterinaria , Divertículo/diagnóstico por imagen , Aracnoides/patología , Aracnoides/diagnóstico por imagen , Relevancia ClínicaRESUMEN
OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.
Asunto(s)
Malformación de Arnold-Chiari , Articulación Atlantooccipital , Atlas Cervical , Hueso Occipital , Fusión Vertebral , Siringomielia , Humanos , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/diagnóstico por imagen , Siringomielia/cirugía , Siringomielia/diagnóstico por imagen , Femenino , Masculino , Atlas Cervical/anomalías , Atlas Cervical/cirugía , Atlas Cervical/diagnóstico por imagen , Niño , Hueso Occipital/cirugía , Hueso Occipital/diagnóstico por imagen , Hueso Occipital/anomalías , Fusión Vertebral/métodos , Adolescente , Articulación Atlantooccipital/diagnóstico por imagen , Articulación Atlantooccipital/cirugía , Articulación Atlantooccipital/anomalías , Resultado del Tratamiento , Preescolar , Descompresión Quirúrgica/métodos , Estudios Retrospectivos , Vértebras Cervicales/cirugía , Vértebras Cervicales/anomalías , Vértebras Cervicales/diagnóstico por imagenRESUMEN
OBJECTIVE: Chiari malformations (CMs) are a group of congenital or acquired disorders characterized by hindbrain overcrowding into an underdeveloped posterior cranial fossa. CM is considered largely sporadic-however, there exists growing evidence of transmissible genetic underpinnings. The purpose of this systematic review of all familial studies of CM was to investigate the existence of an inherited component and provide recommendations to manage and monitor at-risk family members. METHODS: This paper includes the following: 1) a unique case report of dizygotic twins who presented at the Toronto Western Hospital Spinal Cord Clinic with symptomatic CM type 1 (CM-1) and syringomyelia; and 2) a systematic review of familial CM. The EMBASE and MEDLINE databases were searched on June 27, 2023, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Only articles in the English language concerning the diagnosis of CM in > 1 human family member presented as a case study, case series, or literature review were included. RESULTS: Among the 29 articles included in the final analysis, a total of 34 families with CM were analyzed. An average of 3 cases of CM were found per family among all generations. Eighty-one cases (88%) reported CM-1, whereas the other 11 (12%) cases reported either CM-0, CM-1.5, or tonsillar ectopia. A syrinx was present in 37 (54%) cases, with 14 (38%) of these patients also reporting a skeletal abnormality, the most common comorbidity. Most family members diagnosed with CM were siblings (18; 35%), followed by monozygotic twins/triplets (12; 23%). CONCLUSIONS: Patients most often presented with headaches, sensory disturbances, or generalized symptoms. Overall, there exists mounting evidence for a hereditary component of CM. It is unlikely to be explained by a classic mendelian inheritance pattern, but is rather a polygenic architecture influenced by variable penetrance, cosegregation, and entirely nongenetic factors. For first-degree relatives of those affected by CM, the authors' findings may influence clinicians to conduct closer clinical and radiographic monitoring, promote patient education, and consider earlier genetic testing.
Asunto(s)
Malformación de Arnold-Chiari , Humanos , Malformación de Arnold-Chiari/genética , Malformación de Arnold-Chiari/cirugía , Siringomielia/genética , Siringomielia/diagnóstico por imagen , Gemelos Dicigóticos/genéticaRESUMEN
BACKGROUND: Spinal hemangioblastomas are often evaluated with catheter angiography for both workup and treatment planning. We report a unique longitudinal pulse-synchronous bouncing phenomenon observed during their angiographic evaluation and consider the association of pulse-synchronous bouncing with syringomyelia, another pathologic feature associated with hemangioblastomas. METHODS: Preoperative spinal angiograms and associated magnetic resonance imagings (MRIs) obtained over a 16-year period at a single institution were retrospectively evaluated. Magnetic resonance imaging (MRI) parameters included lesion and syrinx location and size. Angiograms were evaluated for bouncing phenomena. Student's t-test and Chi square test compared characteristics between groups. Linear regression analyses evaluated maximum amplitude of dynamic motion and any associated syrinx. RESULTS: Nineteen hemangioblastoma patients had preoperative angiograms available for review. Eight exhibited bouncing behavior. Between the dynamic and nondynamic cohorts, there was no difference in presence or volume of syrinxes. Lesions in the dynamic cohort trended towards a cervical location (75% vs. 36.3%, P = 0.10). No significant correlation was found between bouncing amplitude and syrinx size (R2 = 0.023). Dural contact may be related to this dynamic behavior since other high-flow lesions like AVMs do not demonstrate this phenomenon, and AVMs are pial-based and more likely to contact stationary dura. Here, there were fewer lesions abutting the thecal sac in the dynamic cohort (50% vs. 81.8%, P = 0.14). CONCLUSIONS: Though no significant relationship was established between this bouncing behavior and syrinx formation, noted trends included a greater range of motion for cervical lesions and limited motion in tumors abutting the thecal sac.
Asunto(s)
Hemangioblastoma , Neoplasias de la Médula Espinal , Humanos , Hemangioblastoma/diagnóstico por imagen , Hemangioblastoma/cirugía , Femenino , Masculino , Persona de Mediana Edad , Adulto , Estudios Retrospectivos , Neoplasias de la Médula Espinal/diagnóstico por imagen , Neoplasias de la Médula Espinal/cirugía , Anciano , Imagen por Resonancia Magnética , Siringomielia/diagnóstico por imagen , Siringomielia/etiología , Siringomielia/cirugía , Adulto Joven , Angiografía/métodosRESUMEN
BACKGROUND: Syringomyelia is a progressive chronic disease that leads to nerve pain, sensory dissociation, and dyskinesia. Symptoms often do not improve after surgery. Stem cells have been widely explored for the treatment of nervous system diseases due to their immunoregulatory and neural replacement abilities. METHODS: In this study, we used a rat model of syringomyelia characterized by focal dilatation of the central canal to explore an effective transplantation scheme and evaluate the effect of mesenchymal stem cells and induced neural stem cells for the treatment of syringomyelia. RESULTS: The results showed that cell transplantation could not only promote syrinx shrinkage but also stimulate the proliferation of ependymal cells, and the effect of this result was related to the transplantation location. These reactions appeared only when the cells were transplanted into the cavity. Additionally, we discovered that cell transplantation transformed activated microglia into the M2 phenotype. IGF1-expressing M2 microglia may play a significant role in the repair of nerve pain. CONCLUSION: Cell transplantation can promote cavity shrinkage and regulate the local inflammatory environment. Moreover, the proliferation of ependymal cells may indicate the activation of endogenous stem cells, which is important for the regeneration and repair of spinal cord injury.
Asunto(s)
Trasplante de Células Madre Mesenquimatosas , Células Madre Mesenquimatosas , Células-Madre Neurales , Ratas Sprague-Dawley , Siringomielia , Animales , Células-Madre Neurales/metabolismo , Células-Madre Neurales/citología , Trasplante de Células Madre Mesenquimatosas/métodos , Células Madre Mesenquimatosas/metabolismo , Células Madre Mesenquimatosas/citología , Siringomielia/terapia , Ratas , Proliferación Celular , Epéndimo , Masculino , Microglía/metabolismo , Modelos Animales de EnfermedadRESUMEN
The diagnosis of Chiari I malformation is straightforward in patients with typical signs and symptoms of Chiari I malformation and magnetic resonance imaging (MRI) confirming ≥5 mm of cerebellar tonsillar ectopia, with or without a syrinx. However, in many cases, Chiari I malformation is discovered incidentally on MRI to evaluate global headache, cervical radiculopathy, or other conditions. In those cases, the clinician must consider if cerebellar tonsillar ectopia is related to the presenting symptoms. Surgical decompression of the cerebellar tonsils and foramen magnum in patients with symptomatic Chiari I malformation effectively relieves suboccipital headache, reduces syrinx distension, and arrests syringomyelia progression. Neurosurgeons must avoid operative treatments decompressing incidental tonsillar ectopia, not causing symptoms. Such procedures unnecessarily place patients at risk of operative complications and tissue injuries related to surgical exploration. This chapter reviews the typical signs and symptoms of Chiari I malformation and its variant, Chiari 0 malformation, which has <5 mm of cerebellar tonsillar ectopia and is often associated with syringomyelia. Chiari I and Chiari 0 malformations are associated with incomplete occipital bone development, reduced volume and height of the posterior fossa, tonsillar ectopia, and compression of the neural elements and cerebrospinal fluid (CSF) pathways at the foramen magnum. Linear, angular, cross-sectional area, and volume measurements of the posterior fossa, craniocervical junction, and upper cervical spine identify morphometric abnormalities in Chiari I and Chiari 0 malformation patients. Chiari 0 patients respond like Chiari I patients to foramen magnum decompression and should not be excluded from surgical treatment because their tonsillar ectopia is <5 mm. The authors recommend the adoption of diagnostic criteria for Chiari 0 malformation without syringomyelia. This chapter provides updated information and guidance to the physicians managing Chiari I and Chiari 0 malformation patients and neuroscientists interested in Chiari malformations.
Asunto(s)
Malformación de Arnold-Chiari , Coristoma , Siringomielia , Humanos , Siringomielia/diagnóstico por imagen , Malformación de Arnold-Chiari/complicaciones , Fosa Craneal Posterior , Hueso Occipital , CefaleaRESUMEN
Syringomyelia associated with extensive spinal adhesive arachnoiditis (SAA) can be defined as a rare but progressive disease with potentially devastating clinical consequences. Diagnosis can be challenging due to the absence of specific clinical findings, confusion with other pathologies, and late imaging modalities. The treatment strategy for syringomyelia associated with extensive SAA should include direct drainage of the syringomyelia, and dissection of the adhesive arachnoid with expansive duraplasty. Hence, several approaches have been reported for arachnoid dissection and decompression of the subarachnoid space. The high risk of recurrence after the operation is one of the most challenging situations. First two cases were operated previously, and the cyst walls were removed and anastomosed to the intact subarachnoid space at the upper and lower ends by each other. However, in both cases, the cyst recurred after a short time and they were anastomosed again in our clinic. A subarachnoid-subarachnoid shunt was placed with a multi-hole silicone tube extending to the proximal and distal intact subarachnoid spaces and passing through the cyst removal area. In our third case, the shunt system we described was applied directly, and satisfactory results were obtained in the clinical follow-up of the patient. It has been demonstrated that subarachnoido-subarachnoid shunt is a very satisfactory treatment option for this type of pathology, which is difficult to follow and treat.
Asunto(s)
Quistes Aracnoideos , Aracnoiditis , Espacio Subaracnoideo , Humanos , Quistes Aracnoideos/cirugía , Quistes Aracnoideos/complicaciones , Espacio Subaracnoideo/cirugía , Aracnoiditis/cirugía , Aracnoiditis/etiología , Masculino , Femenino , Siringomielia/cirugía , Siringomielia/etiología , Persona de Mediana Edad , Adulto , Derivaciones del Líquido Cefalorraquídeo/métodos , Recurrencia , Resultado del Tratamiento , Descompresión Quirúrgica/métodos , Imagen por Resonancia Magnética , Procedimientos Neuroquirúrgicos/métodosRESUMEN
We present the case of a man in his 40s who sought medical attention due to central cord syndrome. MRI findings demonstrated contrast uptake, spinal swelling, syrinx formation and narrowing of the spinal canal. We encountered two potential scenarios:when malignancy is suspected, the patient would undergo a biopsy. However, if the lesion is ultimately determined to be benign, the patient would have been subjected to an avoidable risk of neurological damage associated with the procedure. Conversely, addressing the lesion as a result of a degenerative process (discal instability), performing an anterior approach for interbody fusion with an underlying malignant process could lead to substantial delays in the diagnosis, finally producing a poor outcome. A comprehensive imaging workup was conducted to rule out malignancy. We hypothesised that discal instability was responsible for the observed findings. The patient was successfully treated with anterior cervical decompression and fusion, without complications. Follow-up evaluations confirmed remission of the condition.
Asunto(s)
Neoplasias , Enfermedades de la Columna Vertebral , Fusión Vertebral , Siringomielia , Masculino , Humanos , Enfermedades de la Columna Vertebral/patología , Imagen por Resonancia Magnética , Vértebras Cervicales/diagnóstico por imagen , Vértebras Cervicales/cirugía , Vértebras Cervicales/patología , Fusión Vertebral/métodos , Descompresión Quirúrgica/métodos , Resultado del TratamientoRESUMEN
OBJECTIVE: Treatment for Chiari malformation type I (CM-I) often includes surgical intervention in both pediatric and adult patients. The authors sought to investigate fundamental differences between these populations by analyzing data from pediatric and adult patients who required CM-I decompression. METHODS: To better understand the presentation and surgical outcomes of both groups of patients, retrospective data from 170 adults and 153 pediatric patients (2000-2019) at six institutions were analyzed. RESULTS: The adult CM-I patient population requiring surgical intervention had a greater proportion of female patients than the pediatric population (p < 0.0001). Radiographic findings at initial clinical presentation showed a significantly greater incidence of syringomyelia (p < 0.0001) and scoliosis (p < 0.0001) in pediatric patients compared with adult patients with CM-I. However, presenting signs and symptoms such as headaches (p < 0.0001), ocular findings (p = 0.0147), and bulbar symptoms (p = 0.0057) were more common in the adult group. After suboccipital decompression procedures, 94.4% of pediatric patients reported symptomatic relief compared with 75% of adults with CM-I (p < 0.0001). CONCLUSIONS: Here, the authors present the first retrospective evaluation comparing adult and pediatric patients who underwent CM-I decompression. Their analysis reveals that pediatric and adult patients significantly differ in terms of demographics, radiographic findings, presentation of symptoms, surgical indications, and outcomes. These findings may indicate different clinical conditions or a distinct progression of the natural history of this complex disease process within each population, which will require prospective studies to better elucidate.
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Malformación de Arnold-Chiari , Descompresión Quirúrgica , Humanos , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/diagnóstico por imagen , Malformación de Arnold-Chiari/complicaciones , Femenino , Masculino , Niño , Estudios Retrospectivos , Descompresión Quirúrgica/métodos , Adulto , Adolescente , Adulto Joven , Persona de Mediana Edad , Resultado del Tratamiento , Siringomielia/cirugía , Siringomielia/diagnóstico por imagen , Siringomielia/complicaciones , Preescolar , Factores de Edad , Escoliosis/cirugía , Escoliosis/diagnóstico por imagenRESUMEN
OBJECTIVE: This study compared the clinical therapeutic efficacy of syringo-subarachnoid shunt placement with direct tube and T-tube via the dorsal root entry zone (DREZ) approach for treatment of eccentric syringomyelia. METHODS: A retrospective study was performed of 41 patients with idiopathic or secondary eccentric syringomyelia from November 2011 to December 2022. Syringo-subarachnoid shunt placement with direct tube or T-tube via the DREZ approach was performed. The modified Japanese Orthopaedic Association low back pain scale was used to investigate the severity of clinical symptoms. Magnetic resonance imaging was used to investigate therapeutic efficacyï¼reduction of the cavity volume by >10% was considered an improvement and 50% was considered a significant improvement). RESULTS: Incision length of the spinal cortex in the direct tube group was shorter than in the T-tube group (3.10 ± 0.28 cm vs. 5.03 ± 0.19 cm), with a significant difference between the 2 groups (t = -52.56, P < 0.001). Modified Japanese Orthopaedic Association score 3 months postoperatively was significantly better than the preoperative score in both the direct tube groupï¼t = 40.954, P < 0.001ï¼ and the T-tube groupï¼t = 24.769, P < 0.001ï¼. Statistical comparison revealed there was no difference in imaging improvement between the direct tube group and T-tube group 3 months (χ2 = 0.20, P = 0.655) and 12 months (χ2 = 0.21, P = 0.647) postoperatively. CONCLUSIONS: Syringo-subarachnoid shunt placement with direct tube via the DREZ approach for treatment of eccentric syringomyelia is safer than with T-tube via the DREZ approach due to smaller incision length and less of a space-occupying effect with same therapeutic efficacy.
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Derivaciones del Líquido Cefalorraquídeo , Siringomielia , Humanos , Siringomielia/cirugía , Siringomielia/diagnóstico por imagen , Femenino , Masculino , Estudios Retrospectivos , Persona de Mediana Edad , Adulto , Derivaciones del Líquido Cefalorraquídeo/métodos , Resultado del Tratamiento , Raíces Nerviosas Espinales/cirugía , Raíces Nerviosas Espinales/diagnóstico por imagen , Espacio Subaracnoideo/cirugía , Espacio Subaracnoideo/diagnóstico por imagen , Anciano , Imagen por Resonancia MagnéticaRESUMEN
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.
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Articulación Atlantoaxoidea , Enfermedades de los Perros , Siringomielia , Humanos , Perros , Animales , Estudios Retrospectivos , Enfermedades de los Perros/diagnóstico por imagen , Enfermedades de los Perros/epidemiología , Enfermedades de los Perros/congénito , Siringomielia/diagnóstico por imagen , Siringomielia/epidemiología , Siringomielia/veterinaria , Examen Físico , Peso Corporal , Articulación Atlantoaxoidea/diagnóstico por imagenRESUMEN
BACKGROUND: Syringomyelia (SM) is characterized by the development of fluid-filled cavities, referred to as syrinxes, within the spinal cord tissue. The molecular etiology of SM post-spinal cord injury (SCI) is not well understood and only invasive surgical based treatments are available to treat SM clinically. This study builds upon our previous omics studies and in vitro cellular investigations to further understand local fluid osmoregulation in post-traumatic SM (PTSM) to highlight important pathways for future molecular interventions. METHODS: A rat PTSM model consisting of a laminectomy at the C7 to T1 level followed by a parenchymal injection of 2 µL quisqualic acid (QA) and an injection of 5 µL kaolin in the subarachnoid space was utilized 6 weeks after initial surgery, parenchymal fluid and cerebrospinal fluid (CSF) were collected, and the osmolality of fluids were analyzed. Immunohistochemistry (IHC), metabolomics analysis using LC-MS, and mass spectrometry-based imaging (MSI) were performed on injured and laminectomy-only control spinal cords. RESULTS: We demonstrated that the osmolality of the local parenchymal fluid encompassing syrinxes was higher compared to control spinal cords after laminectomy, indicating a local osmotic imbalance due to SM injury. Moreover, we also found that parenchymal fluid is more hypertonic than CSF, indicating establishment of a local osmotic gradient in the PTSM injured spinal cord (syrinx site) forcing fluid into the spinal cord parenchyma to form and/or expand syrinxes. IHC results demonstrated upregulation of betaine, ions, water channels/transporters, and enzymes (BGT1, AQP1, AQP4, CHDH) at the syrinx site as compared to caudal and rostral sites to the injury, implying extensive local osmoregulation activities at the syrinx site. Further, metabolomics analysis corroborated alterations in osmolality at the syrinx site by upregulation of small molecule osmolytes including betaine, carnitine, glycerophosphocholine, arginine, creatine, guanidinoacetate, and spermidine. CONCLUSIONS: In summary, PTSM results in local osmotic disturbance that propagates at 6 weeks following initial injury. This coincides with and may contribute to syrinx formation/expansion.
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Traumatismos de la Médula Espinal , Siringomielia , Ratas , Animales , Siringomielia/etiología , Osmorregulación , Betaína , Ratas Sprague-Dawley , Traumatismos de la Médula Espinal/complicaciones , Traumatismos de la Médula Espinal/metabolismo , Imagen por Resonancia MagnéticaRESUMEN
BACKGROUND: Chiari malformations are a rare group of rhomboencephalic abnormalities involving the brain, craniocervical junction and spine. They may manifest in a variety of clinical presentations which relate to the variable involvement of the cerebellum, brainstem, lower cranial nerves, spinal cord and altered CSF flow dynamics. METHOD: We report an unusual case of incidental diagnosis of a type I Chiari malformation with secondary cystic cerebellar tonsillar encephalomalacia and holocord syrinx following investigation of a 5YO girl presenting with heel swelling related to progressive neuropathic osteoarthropathy of the posterior calcaneal body and apophysis. RESULT: The child was treated with decompressive suboccipital craniectomy and C1 laminectomy and tonsillar resection. Cerebellar tonsillar gliosis and cystic degeneration were confirmed on histopathology. Referral for ongoing engagement with occupational and physical therapy. CONCLUSION: Most type I Chiari malformations in the paediatric population are incidental and asymptomatic. Neurological symptoms are typically mild and relate to altered CSF flow dynamics; however, we present a complex case of type I Chiari malformation with an unusual constellation of associated complications.