Syringomyelia: Retrospective Analysis of 30 Surgically Treated Patients from the Northeast of Brazil / Siringomielia: Análise retrospectiva de 30 casos operados no nordeste brasileiro

Introduction Syringomyelia is a chronic disease characterized by the presence of intramedullary cavity. Chiari malformation (CM) and basilar impression (BI) are conditions usually associated with syringomyelia. Its prevalence has wide geographical variation, being higher in the Northeast of Brazil, making it relevant to study the subject in this region. Objective To analyze the frequency of signs, symptoms, and surgical aspects observed in patients undergoing decompressive treatment. Methods We performed a retrospective analysis of the medical records of patients diagnosed with syringomyelia who received decompressive surgical treatment in various hospitals in João Pessoa, Paraíba, between 1994 and 2021. Results Thirty patients were analyzed. Twenty-nine (96.7%) presented CM and 27 (90.0%) also presented BI. A wide variety of symptoms was found, with significant prevalence of muscle weakness, neck pain, and headache. Brevicollis, a finding considered typical of the Northeastern region and associated with craniocervical junction malformations, was present in 66.7%. The surgical technique used in 90% of patients was similar. Fourteen (46.7%) patients presented difficult craniocervical junction and 4 (13.3%) had occipital bone assimilation. Eighteen (60.0%) presented thickening of the arachnoid membrane. Postoperatively, there was clinical improvement in 21 patients (70%). Conclusions The sample majorly had CM and BI associated with syringomyelia. High prevalence of signs and symptoms related to the Brazilian northeastern phenotype was also found. Syringomyelia, therefore, has peculiarities in the population of the Northeast of Brazil that, when described, allow better understanding of the pathology in this group.

Introdução Siringomielia é uma enfermidade crônica caracterizada pela presença de cavidade intramedular. Costuma vir associada a condições como malformação de Chiari (MC) e impressão basilar (IB). Sua prevalência tem grande variação geográfica, sendo maior no nordeste brasileiro, o que torna relevantes estudos sobre o tema nessa região. Objetivo Analisar a frequência de sinais, sintomas e aspectos cirúrgicos observados em pacientes submetidos a tratamento descompressivo. Método Foi realizada análise retrospectiva das informações contidas nos prontuários de pacientes diagnosticados com siringomielia submetidos a tratamento cirúrgico descompressivo em vários hospitais de João Pessoa, Paraíba, entre 1994 e 2021. Resultados Foram analisados 30 pacientes, dos quais 29 (96,7%) apresentaram MC associada e 27 (90,0%) também tinham IB. Houve grande variedade de sintomas, com importante prevalência de fraqueza muscular, cervicalgia e cefaleia. Brevicolia, um achado considerado típico do nordestino e associado a malformações da junção craniocervical, esteve presente em 66,7% dos pacientes. A técnica cirúrgica utilizada em 90% dos pacientes foi semelhante. Quatorze (46,7%) pacientes apresentaram junção crânio-cervical difícil; e quatro (13,3%) apresentaram assimilação do osso occipital. Dezoito (60,0%) apresentaram espessamento da membrana aracnoide. No pós-operatório, houve melhora clínica em 21 pacientes (70%). Conclusão A expressiva maioria da amostra possuía MC e IB associadas à siringomielia. Nota-se também grande prevalência de sinais e sintomas relacionados ao fenótipo nordestino. A siringomielia, portanto, possui peculiaridades na população do Nordeste brasileiro que, ao serem descritas, permitem a melhor compreensão da patologia nesse perfil de pacientes.

Charcot Arthropathy of the Shoulder Associated with Syringomyelia: A Report of 2 Cases / Artropatia do ombro de Charcot associada à Siringomielia: Relato de 2 casos

Charcot arthropathy of the shoulder caused by syringomyelia is a unusual degenerative disorder, frequently misdiagnosed and with few cases described in the literature. The diagnosis is made by clinical evaluation and radiological examinations with radiography and magnetic resonance imaging. However, the correct diagnosis and treatment is possible by carefully medical evaluation and can improve patient symptoms. Therefore, this study aimed to report two cases of Charcot arthropathy caused by syringomyelia. After achieving correct neurosurgical evaluation and magnetic resonance imaging, the diagnosis was made. The first case is a 53-year-old man with a click on his right shoulder for at least 12 months, associated with local edema, pain and limitation of joint range of motion. The second is a 45-year-old man with pain in the right upper limb and difficulty moving the joint for at least 24 months, associated with progressive worsening of the collection and edema in the ipsilateral upper limb. Posterior fossa decompression was performed, with symptoms relief after surgery. Posterior fossa decompression is a treatment that seems to be effective in reducing symptoms, especially when the diagnosis is early. However, this type of treatment still remains controversial, requiring further studies.

A artopatia de Charcot do ombro causada por siringomielia é uma doença degenerativa incomum, frequentemente subdiagnosticada e com poucos casos descritos na literatura. O diagnóstico é feito pela avaliação clínica e exames radiológicos com radiografia e ressonância magnética. No entanto, o diagnóstico e tratamento corretos são possíveis mediante avaliação médica criteriosa e podem melhorar os sintomas do paciente. Portanto, este trabalho objetiva relatar dois casos de artropatia de Charcot causada por siringomielia. Após obter correta avaliação neurocirúrgica e ressonância magnética, o diagnóstico foi feito. O primeiro caso é um homem de 53 anos com clique no ombro direito por pelo menos 12 meses, associado a edema local, dor e limitação da amplitude do movimento articular. O segundo é um homem de 45 anos com dor em membro superior direito e dificuldade de movimentação articular há pelo menos 24 meses, associada a piora progressiva da coleção e edema em membro superior ipsilateral. A descompressão da fossa posterior foi realizada, com alívio dos sintomas após a cirurgia. A descompressão da fossa posterior é um tratamento que parece eficaz na redução dos sintomas, principalmente quando o diagnóstico é precoce. Porém, esse tipo de tratamento ainda permanece controverso, necessitando de mais estudos.

Descompresión de fosa posterior sin duroplastia expansiva en adulto con malformación de Chiari tipo 1-siringomielia

Introducción: La malformación de Chiari tipo 1 incluye un grupo heterogéneo de malformaciones congénitas, caracterizadas por el descenso caudal del cerebelo a través del foramen magno. En un 30-70 % de los casos tiene siringomielia asociada. Existen controversias en torno a la técnica quirúrgica ideal. Objetivo: Presentar un caso de MC-1 asociada a siringomielia en el que no se aplica una duroplastia expansiva. Presentación de caso: Paciente femenina de 43 años, con antecedentes de hipertensión arterial. Acudió a consulta neuroquirúrgica por dolor cervical irradiado al miembro superior derecho. Al examen neurológico mostró signos de afectación de primera y segunda motoneurona. La resonancia magnética confirmó el diagnóstico de MC-1. Se intervino quirúrgicamente mediante descompresión de fosa posterior sin duroplastia expansiva. La paciente evolucionó sin complicaciones y egresó a las 48 horas. Durante el seguimiento mejoraron las manifestaciones parestésicas; sin embargo, el examen neurológico se mantuvo igual. A los seis meses, la resonancia magnética indicó una marcada disminución de la siringomielia y la reconformación de la cisterna magna. Hasta los 18 meses del tratamiento, los síntomas no habían empeorado y la capacidad funcional resultaba aceptable (Karnofsky 90/100). Conclusiones: La descompresión de fosa posterior sin duroplastia expansiva, seguida de re-permeabilización microquirúrgica del foramen de Magendie, tuvo resultados favorables en este caso.

Introduction: Chiari malformation type 1 includes a heterogeneous group of congenital malformations, characterized by caudal descent of the cerebellum through the foramen magnum. It has associated syringomyelia in 30-70% of cases. Controversies exist regarding the ideal surgical technique. Objective: To present a case of MC-1 associated with syringomyelia in which an expansive duroplasty is not applied. Case report: Female patient, 43 years old, with history of arterial hypertension. She went to the neurosurgical consultation for cervical pain radiating to the right upper limb. On neurological examination, she showed signs of first and second motor neuron involvement. MRI confirmed the diagnosis of MC-1. She underwent surgery by decompression of the posterior fossa without expansive duroplasty. The patient evolved without complications and she was discharged after 48 hours. During the follow-up, the paresthetic manifestations improved; however, the neurological examination remained the same. At six months, MRI indicated a marked decrease in syringomyelia and reshaping of the cisterna magna. Until 18 months after treatment, symptoms had not worsened and functional capacity was acceptable (Karnofsky 90/100). Conclusions: Posterior fossa decompression without expansive duroplasty, followed by microsurgical re-permeabilization of Magendie's foramen, had favorable results in our case.

Utilidad práctica de la dinámica cuantitativa de LCR en la malformación de Chiari tipo I y la siringomielia. Reporte de 5 casos / Practical utility of quantitative CSF dynamics in type I Chiari malformation and syringomyelia. Report of 5 cases

Introducción: el tratamiento de la malformación de Chiari I (MCI) y/o la siringomielia (SM) es controversial. La dinámica cuantitativa del LCR a nivel cráneo espinal es una alternativa que podría orientar la terapéutica. El objetivo de esta publicación es describir 5 casos en donde la utilización de la dinámica de LCR permitió guiar el tratamiento. Material y método: se revisaron las historias clínicas de 5 casos (edad media: 39 años / 3 mujeres y 2 varones). Todos fueron estudiados con RM en contraste de fase. El diagnóstico fue de MCI (1 caso) y SM (3 casos) o solo SM (1 caso). Sólo 2 casos con MCI+SM fueron intervenidos (descompresión + plástica dural). Todos fueron seguidos entre 1,5 y 6 años. Resultados: caso 1 (MCI) la velocidad del LCR fue normal por lo que su cefalea fue tratada médicamente con buenos resultados; caso 2 (MCI+SM) la velocidad estuvo aumentada por lo que fue intervenida controlándose los síntomas y la SM; caso 3 (MCI +SM) la velocidad fue normal siendo su diagnóstico compatible con síndrome post-siringomiélico; caso 4 (SM) la velocidad estuvo aumentada a nivel C5-C6 siendo su diagnóstico compatible con una SM espinal primaria; caso 5 (CMI + SM) luego de la intervención se observó que las velocidades y la SM tardaron 16 meses en normalizarse. Conclusión: en los casos descriptos la dinámica de LCR permitió realizar el diagnóstico correcto, determinar la conveniencia de realizar la cirugía, encontrar la causa y controlar la evolución postoperatoria(AU)

Background: the treatment of Chiari malformation I (CMI) and/or syringomyelia (SM) is controversial. The quantitative dynamics of CSF at the craniospinal level is an alternative that could guide therapy. The objective of this publication is to describe 5 cases in which the use of CSF dynamics allowed guiding the treatment. Methods: the medical records of 5 cases (mean age: 39 years / 3 women and 2 men) were reviewed. All were studied with MRI in phase contrast. The diagnosis was CMI (1 case) and SM (3 cases) or only SM (1 case). Only 2 cases with CMI+SM underwent surgery (decompression + duraplasty). All were followed between 1.5 and 6 years. Results: case 1 (CMI) the velocity of the CSF was normal, so his headache was treated medically with good results; case 2 (CMI+SM) the velocity was increased so it was intervened controlling the symptoms and the SM; case 3 (CMI +SM) the velocity was normal, its diagnosis being compatible with post-syringomyelic syndrome; case 4 (SM) the velocity was increased at the C5-C6 level, its diagnosis being compatible with a primary spinal SM; case 5 (CMI + SM) after the intervention it was observed that the velocities and the SM took 16 months to normalize. Conclusion: in the cases described, the CSF dynamics allowed the correct diagnosis to be made, to determine the advisability of performing surgery, to find the cause and to control the postoperative evolution(AU)

A rare case of Syringomyelia with Arnold � Chiari Malformation Type 1 - A case report

Syringomyelia is a pathologic cystic cavity within the spinal cord containing cerebrospinal fluid (CSF). It is commonly seen as a complication of an Arnold-Chiari type 1 malformation, which is the herniation of cerebellar tonsils through foramen magnum into cervical spinal canal. Syringomyelia can also occur as complication of hemorrhage, tumor, meningitis, arachnoiditis, or trauma. Symptoms usually begin to appear in early and middle life. These symptoms usually consist of pain, dissociated sensory loss and weakness that present and progress gradually. We herewith report a rare case of syringomyelia and associated Chiari I malformation presenting with dissociated sensory impairment in neck region with headache and neck pain Treatment in these cases is surgical decompression. Recovery with significant decrease in sensory loss and relief in headache and neck pain.

Research progress of Chiari malformation type I and atlantoaxial instability / 中华骨科杂志

Chiari malformation (CM) is a group of congenital cerebellar tonsillar hernia malformations involving the craniocervical junction. Chiari malformation type I (CMI) is the most common in clinic, however its pathogenesis is still unclear, and there is no consensus on the surgical treatment standard of CMI. At present, the most widely accepted is the theory of posterior fossa incompatibility, so doctors at home and abroad use posterior fossa decompression (PFD) and posterior fossa compression with duraplasty (PFDD) as the gold standard for surgical treatment, and have their own experience and technical improvement. However, the volume of the posterior cranial fossa in some patients is no different from that in healthy people, and about 30% of the patients with CMI have poor results after posterior cranial fossa decompression. As a result, this operation cannot treat all patients with CMI. In recent years, with the development of imaging, the progress of diagnostic technology and the deepening of understanding of CM, some studies have shown that CMI may be related to atlantoaxial instability, and proposed that CMI is the secondary factor of atlantoaxial instability, and atlantoaxial fusion is the standard of surgical treatment, which has caused great controversy in academic circles. Different clinical research results of scholars support or oppose this theory: some studies have shown that the clinical symptom relief rate of patients with CMI treated with atlantoaxial fusion is 96.9%; another study showed that 70% of patients with CMI underwent atlantoaxial fusion had improved neurological function, but the overall postoperative effect was not satisfactory. In short, CMI is related to many diseases and its clinical manifestations are complex. Therefore, individualized management and treatment should be carried out in combination with the clinical manifestations and auxiliary examination results of patients.

Siringomielia: revisión a propósito de un caso / Syringomyelia: review, case and report

La siringomielia supone un desafío diagnóstico, ya que es una entidad poco reconocida si no se tiene conciencia de su existencia. Al ser un cuadro progresivo, cuya clínica puede presentarse de forma larvada y ser coincidente con otras patologías neurológicas tales como la esclerosis múltiple, su detección suele realizarse en etapas tardías sobre todo en población adulta y más aún cuando se presenta de forma adquirida. Por lo que el estudio imagenológico con Resonancia Magnética adquiere especial relevancia, permitiendo identificar y clasificar la enfermedad, lo que brindará la base para decidir terapía.

Syringomyelia is a diagnostic challenge, since it is a poorly recognized disease, especially if its existence remains unknown. Being a progressive disease, whose clinic can present in a latent way and be coincident with other neurological pathologies such as multiple sclerosis, its detection is usually conducted in late stages, especially in the adult population and even more when it presents in an acquired way. Therefore, the imaging study with Magnetic Resonance acquires special relevance, allowing to be identified and classified, which will provide the basis for deciding on therapy

Lesión difusa de médula espinal, ¿cirugía o tratamiento paliativo?: a propósito de un caso

Las lesiones difusas de médula son raras y un reto diagnóstico. Los gliomas de bajo grado son los de mayor prevalencia y los astrocitomas primarios, el subtipo más frecuente. Presentamos el caso de un varón de 36 años, con tiempo de enfermedad de 13 años de dolor cervical insidioso, progresa con hemiparesia derecha y, posteriormente, paraparesia severa con pérdida de control de esfínteres. Mediante una RMN total de columna se evidenciaron lesiones neoproliferativas difusas abarcando todos los segmentos medulares. Se realizó una biopsia a cielo abierto en segmento T1-T2, la patología diagnosticó glioma de bajo grado. El tratamiento es controversial y se descartó la cirugía por la alta morbilidad. Se optó por radioterapia y quimioterapia por su rol importante en el control de la enfermedad.

Diffuse spinal cord injuries are rare and pose a diagnostic challenge. Low-grade gliomas are the most prevalent type, with primary astrocytomas being the most frequent subtype. We present the case of a 36-year-old man with a 13-year history of squeezing neck pain, which progressed with right hemiparesis and later with severe paraparesis and loss of sphincter control. A whole spine MRI scan showed diffuse neoproliferative lesions that spread throughout all the spinal cord segments. An open biopsy of the lesion in the T1-T2 segment was performed. The pathology report stated low-grade glioma. Treatment is controversial, so surgery was ruled out due to high morbidity. Radiotherapy and chemotherapy were chosen because they play an important role in controlling the disease.

The clinical effect of posterior fossa decompression combined with dural reconstruction in the treatment of Chiari malformation-Ⅰ complicated with syringomyelia / 中国医师进修杂志

Objective:To investigate the clinical effect of posterior fossa decompression combined with dural reconstruction in the treatment of Chiari malformation-Ⅰ(CM-Ⅰ) complicated with syringomyelia (SM).Methods:The clinical data of 50 patients with CM-Ⅰ complicated with SM who were treated in Yan′an University Xianyang Hospital from June 2019 to January 2021 were analyzed. They were divided into the study group (27 cases) and the control group (23 cases) according to the surgical methods. The former received posterior fossa decompression combined with dural reconstruction, while the latter received posterior fossa decompression alone. The clinical symptom improvement, neurological function, cerebrospinal fluid dynamics and syringomyelia changes were compared between the two groups before and after the surgery, and postoperative complications were compared.Results:The overall clinical symptom improvement rate between the two groups had no significant difference ( P> 0.05). After the surgery, the scores of pain, sensory disturbance, dyskinesia and ataxia in the study group were higher than those in the control group: (4.56 ± 0.35) points vs. (4.28 ± 0.43) points, (3.61 ± 0.82) points vs. (3.15 ± 0.73) points, (3.81 ± 0.44) points vs. (3.59 ± 0.50) points, (4.43 ± 0.41) points vs. (4.09 ± 0.53) points, there were statistical significant ( P<0.05). After the surgery, the cerebrospinal fluid stroke volume (SV) and mean flow (MF) in the study group were higher than those in the control group: (0.05 ± 0.02) ml vs. (0.04 ± 0.01) ml, (0.05 ± 0.01) ml/s vs. (0.04 ± 0.01) ml/s; the maximum peak flow velocity (V max) of the head and tail in the study group were lower than those in the control group: (3.14 ± 1.05) mm/s vs. (3.87 ± 1.13) mm/s, (5.56 ± 1.38) mm/s vs. (6.43 ± 1.22) mm/s, there were statistical significant ( P<0.05). There were no significant differences in the rate of reduction or disappearance of syringomyelia, the rate of no change and the rate of increase of syringomyelia after the surgery between the two groups ( P>0.05). There was no significant difference in the incidence of postoperative complications between the two groups ( P>0.05). Conclusions:Posterior fossa decompression combined with dural reconstruction in CM-Ⅰ complicated with SM can better improve cerebrospinal fluid dynamics, and promote the reduction of syringomyelia without increasing postoperative complications.

Imaging study of simple Chiari malformation type I and atlantoaxial instability / 中华骨科杂志

Objective:To investigate the relationship between simple Chiari malformation type I (CMI) and atlantoaxial instability from the imaging point of view.Methods:A retrospective analysis were performed on 46 patients diagnosed with simple CMI from January 2014 to December 2020. Forty-six normal people matched for age and sex were selected as the normal control group, while 30 patients with atlantoaxial dislocation were selected as the dislocation group. The degree of atlantoaxial joint degeneration in each group was assessed according to Weishaupt degeneration grading; the atlantoaxial joint angulation angle was measured in the control group of patients with simple CMI; and the sagittal imaging parameters of cervical spine X-ray were measured, including C 0-C 1 Cobb angle, C 0-C 2 Cobb angle, C 1-C 2 Cobb angle, C 1-C 7 Cobb angle, C 2-C 7 Cobb angle, C 7 Slope, C 2 Tilt, spino cranial angle (SCA), and C 2-C 7 sagittal vertebral axis (SVA). All radiographic parameters were measured twice independently by two spine surgeons, and intraclass correlation coefficient (ICC) were determined to demonstrate intra- and inter-observer reliability. Results:ICC ranged between 0.842 and 0.974 in the current study, demonstrating "excellent" reliability of radiographic measurements. No significant difference was noted regarding age and the distribution of genders among the three groups. There were significant differences in the distribution of Weishaupt degeneration grading of atlantoaxial joints between simple CMI, normal and dislocation group ( H=53.68, P<0.001 on the left side; H=43.39, P<0.001 on the right side). There were significant differences in the degree of atlantoaxial joint degeneration between the normal group and dislocation group (left, Z=6.60, P<0.001; right, Z=6.29, P<0.001); There were significant differences in the degree of atlantoaxial joint degeneration between the normal group and simple CMI patients (left, Z=5.31, P<0.001; right, Z=4.13, P<0.001); There were significant differences in the degree of atlantoaxial joint degeneration between simple CMI and dislocation group (left, Z=3.20, P=0.001; right, Z=3.15, P=0.002). There were significant difference in the angulation angle of the atlantoaxial articular surface between the normal group and simple CMI patients (left, Z=3.32, P<0.001; right, Z=5.74, P<0.001). There were significant differences in C 0-C 1 Cobb angle ( t=2.41, P=0.018), C 1-C 7 Cobb angle ( t=2.88, P=0.005), C 2-C 7 Cobb angle ( t=3.29, P=0.001), and C 2-C 7 SVA ( t=2.87, P=0.005) between the normal group and simple CMI patients, but there was no significant difference in other parameters. Conclusion:The degree of atlantoaxial joint degeneration in patients with simple CMI is higher than that in normal people, the angulation angle is larger, and the cervical lordosis is larger, suggesting that there may be atlantoaxial joint instability. This study provides further evidence that Chiari malformation type I is associated with atlantoaxial instability.

Diagnosis and treatment of Chiari malformation and syringomyelia in adults: an interpretation of international consensus document (2021) / 中华神经医学杂志

Chiari malformation (CM) is the most common cause of syringomyelia, where agreed criterions on classification and treatment are still missing. In 2019, 29 international experts from Europe achieved a consensus on the definition, classification, diagnosis and treatment of CM and syringomyelia in adults, aiming to guide the clinical diagnosis and treatment of these diseases. Now the consensus is interpreted based on recently published literature at home and abroad, aiming to provide references for standardized diagnosis and treatment of CM and syringomyelia in adults.

Diagnosis and treatment of Chiari malformation and syringomyelia in adults: an interpretation of international consensus document (2021) / 中华神经医学杂志

Chiari malformation (CM) is the most common cause of syringomyelia, where agreed criterions on classification and treatment are still missing. In 2019, 29 international experts from Europe achieved a consensus on the definition, classification, diagnosis and treatment of CM and syringomyelia in adults, aiming to guide the clinical diagnosis and treatment of these diseases. Now the consensus is interpreted based on recently published literature at home and abroad, aiming to provide references for standardized diagnosis and treatment of CM and syringomyelia in adults.

Siringomielia por malformación de Chiari: resolución espontánea. Revisión bibliográfica / Syringomyelia due to Chiari malformation: spontaneous resolution. Bibliographic review

Objetivo: describir y analizar los casos de siringomielia por malformación de Chiari tipo 1 con resolución espontánea. Método: se buscó en Pubmed bajo las palabras clave malformación de Chiari tipo 1, siringomielia y resolución espontánea todos los casos relacionados. Se registraron las características poblacionales de cada caso, evolución y mecanismos propuestos. Resultados: En la población adulta se encontraron 22 casos (edad media: 35,81) y en la población pediátrica se encontraron 37 casos (edad media: 9,82). Las hipótesis sobre los mecanismos que produjeron la resolución espontánea fueron: cambios en la posición de las amígdalas relacionadas con la edad, atrofia del cerebelo, fisura medular, reducción del esfuerzo físico, variaciones de la presión intraespinal, crecimiento del cráneo y disminución de la presión intracraneana. Conclusión: La resolución espontánea es posible en pocos casos. Si bien este hecho invita a realizar controles periódicos y evitar la cirugía, la espera conlleva riesgos ante la posibilidad de un agravamiento agudo o de que surjan secuelas permanentes. La información disponible hoy en día no permite predecir con seguridad la evolución de cada caso

Objective: describe and analyze the cases of syringomyelia caused by Chiari type 1 malformation with spontaneous resolution. Method: we searched in Pubmed with the key words Chiari malformation type 1, syringomyelia and spontaneous resolution all related cases. The population characteristics of each case were recorded along with its outcome and proposed mechanisms. Results: in the adult population there were 22 cases (medium age: 35.81) and in the pediatric population there were 37 cases (medium age: 9.82). The hypothesis about the mechanisms that produce the spontaneous resolution were: changes in tonsils position related to age, cerebellar atrophy, cord fissures, reduction of physical effort, variations in intraspinal pressure, skull growth and decrease in intracranial pressure. Conclusion: the spontaneous resolution of syringomyelia invites to carry out regular check-ups and avoid surgery. However, waiting carries risks with the possibility of acute worsening or permanent sequelae. The information available today does not allow to predict with certainty the evolution of each case.

Spinal cord atrophy-like changes in adolescent Chiari malformation type I patients: a neglected imaging change / 中华骨科杂志

Abstract:To compare the morphology of spinal cord between healthy adolescents with adolescent Chiari malformation type I (CMI) patients and investigate the impact of syringomyelia on the morphology of spinal cord in CMI patients.Methods:The clinical and radiological data of 292 CMI patients diagnosed by our center between June 2012 and March 2019 were retrospectively reviewed. Among them 15 CMI patients without syringomyelia were recruited in the CM group. Among the remaining 277 CMI patients, 274 patients had syringomyelia below the C 3-4 intervertebral disc. According to the principle of best matching, CMI patients with syringomyelia were selected with the closest age to the CM group (±18 months), and 30 CMI patients with syringomyelia were included in the CMS group according to a ratio of 1∶2. Thirty healthy adolescents were enrolled as the control group (NC group) in the same way. The anteroposterior diameters of spinal cord at C 2 (DSCO-C 2), spinal canal at C 2 (DSCA-C 2), midbrain-pontine junction (DPJ), the distance between the tip of cerebellar tonsils and the foramen magnum (AB) and the maximal diameter of the syrinx (D-syrinx) were measured on MRI. All radiographic parameters were measured twice independently by two spine surgeons, and intraclass correlation coefficient (ICC) were determined to demonstrate intra- and inter-observer reliability. One-way ANOVA and SNK- q test were used to compare the above radiographic parameters and age between CM, CMS and NC group. The distribution of genders was compared between the three groups using Chi-square tests. Pearson correlation analysis were conducted to demonstrate the relationship between radiographic parameters in CM and CMS group. Results:ICC ranged between 0.91 and 0.95 in the current study, demonstrating "excellent" reliability of radiographic measurements. No significant difference was noted regarding age and the distribution of genders among the three groups. Patients in CM and CMS groups showed similar DSCO-C 2 values ( P=0.254), both of which were significantly lower than that in NC group ( P<0.001). DSCA-C 2 in CMS group was significantly larger than that in CM ( P=0.003) and NC ( P<0.001) groups, while no significant difference was found between the CM and NC groups ( P=0.216). Moreover, DPJ in CMS group was significantly lower than that in CM group ( P<0.001) and NC group ( P<0.001). There was no significant difference in AB between CM and CMS groups ( P=0.948). DSCO-C 2 was significantly positively correlated with DSCA-C 2 in CMS group ( r=0.906, P<0.001), while AB, D-syrinx, DSCO-syrinx, DSCA-syrinx and DPJ were not significantly correlated with DSCA-C 2. There were significant correlations observed between DPJ and other radiographic parameters in the CMS group (all P>0.05). Significant positive correlation between DSCO-C 2 and DPJ was observed in CM group ( r=0.703, P=0.005). There was no significant correlation between DSCO-C 2 and DSCA-C 2 and DPJ in NC group (all P>0.05). Conclusion:CMI adolescents have significant atrophic change of cervical spinal cord and midbrain-pontine junction compared with healthy adolescents, regardless of the existence of syrinx. Moreover, syrinx in CMI patients indicated more obvious atrophic change of midbrain-pontine junction and dilated spinal canal compared with isolated CMI patients.

Clinical effects of syringo-pleural shunt in the treatment of syringomyelia / 西安交通大学学报(医学版)

【Objective】 To observe the clinical effects of syringo-pleural shunt as a supplementary in the treatment of syringomyelia. 【Methods】 Eleven patients with syringomyelia were treated with syringo-pleural shunt from December 2017 to December 2019. Tator’s postoperative evaluation standard was used to determine the clinical effects of improvement of clinical symptoms. MRI and the Japanese Orthopedic Association (JOA) score were used to evaluate the improvement of syringomyelia and neurological function. 【Results】 After 3 months, 6 months and 12 months of follow-up, the clinical symptoms and JOA score improved in 10 cases, and 1 case had no improvement and developed severe pleural effusion. MRI showed that syringomyelia was significantly improved in 10 cases, and there was no improvement in 1 case. 【Conclusion】 Syringo-pleural shunt is a safe, direct and effective supplementary treatment method for patients with syringomyelia.

Siringomielia asociada a malformación de Chiari tipo I. Informe de caso / Syringomyelia associated with Chiari type I malformation. Case report

RESUMEN La siringomielia es una cavidad quística de la medula espinal, y se considera un padecimiento progresivo y degenerativo. Existen múltiples y variadas maneras de clasificarla, basadas principalmente en su origen, o en los mecanismos conocidos de producción. Se presenta el caso de un paciente de 27 años, con cuadro de deficiencia motriz de cuatro meses de evolución, que comenzó en miembros superiores y continuó con torpeza a la marcha. Al examen físico presentó marcha paretoespástica, cuadriparesia con predominio de debilidad en miembros superiores con respecto a los inferiores, además de espasticidad crural y braquial izquierda, con hipotonía braquial derecha, asociado con atrofia distal de ambos miembros superiores e hipoestesia térmica y dolorosa suspendida del miembro superior derecho. La resonancia magnética nuclear mostró cavidad siringomiélica amplia en columna cervical, con descenso de las amígdalas cerebelosas, compatible con malformación de Chiari tipo I. Aunque la asociación de ambas entidades es común, el déficit motriz progresivo en un paciente joven es motivo de ingreso o consulta poco frecuente en el Servicio de Neurología. En estos casos, el tratamiento descompresivo puede disminuir el tamaño de la cavidad siringomiélica.

ABSTRACT Syringomyelia is a cystic cavity of the spinal cord, and is considered a progressive and degenerative condition. There are multiple and varied ways to classify it, based mainly on its origin, or on known production mechanisms. A 27-year-old patient with a four-month history of motor impairment, which began in the upper limbs and continued with clumsy gait, was presented. On physical examination, he presented paretospastic gait, quadriparesis with a predominance of weakness in the upper limbs with respect to the lower ones, as well as crural and left brachial spasticity, with right brachial hypotonia, associated with distal atrophy of both upper limbs and suspended thermal and painful hypoesthesia of the limb upper right. Nuclear magnetic resonance imaging showed a wide syringomyelic cavity in the cervical spine, with descent of the cerebellar tonsils, compatible with type I Chiari malformation. Although the association of both entities is common, progressive motor deficit in a young patient is a reason for admission or consultation rare in the Neurology Service. In these cases, decompressive treatment can decrease the size of the syringomyelic cavity.

Polyarticular charcot: A rare case report and a literature review in Indian context

Charcot or neuropathic arthropathies are a progressive form of destructive, erosive and generally painless arthropathies.Prevalence of neuropathic joints has decreased globally with reduction in the cases of leprosy and syphilis. However,syringomyelia and diabetes mellitus have emerged as the major causes for upper limb and lower limb Charcot jointsrespectively. Literature evidence shows lack of India data pertaining to these arthropathies. The present study describesa case of polyarticular Charcot in a patient with syrinx and Chiari malformation. The patient history revealed a provisionaldiagnosis of rheumatoid arthritis and Koch’s elbow, and was treated with anti-tubercular treatment (ATT) and diseasemodifying anti-rheumatic drugs (DMARDS). Nervous system examination would have easily led to the diagnosis ofsyringomyelia. The present study also provides a review of Indian literature on neuropathic joints from 2001 to 2019.Diabetes mellitus, syringomyelia, leprosy and syphilis are major etiologies for Charcot joints.

The use of magnetic resonance phase-contrast cine in Chiari malformation with syringomyelia

@#mesodermal origin and is commonly associated withsyringomyelia. Foramen magnum decompression is thefirst-line of standard treatment in symptomatic patients witha confirmed radiographic diagnosis. Magnetic resonance(MR) cine allows accurate evaluation of cerebrospinal fluid(CSF) physiology at the craniovertebral junction but oftenthis is under-utilised in Malaysia. Methods: In this series, we looked into nine cases of CM withsyringomyelia from clinical and radiological perspectivebefore and after surgery. The radiological parameters wereherniated tonsillar length, syrinx: cord ratio, syrinx lengthand diameter. Flow velocity and morphologic changes inChiari were illustrated. Results: Seven patients showed either reduction in syrinxlength, syrinx: cord ratio or both postoperatively. Clinicalrecovery somewhat varied in motor and sensory symptoms.Four patients gained better functional grade in modifiedRankin scale (MRS) while the rest remained similar. Thestudy highlighted the advantage of CSF flow dynamicsinformation over MR anatomical radiographic improvementin addressing the neurologic and functional recovery. Wealso discussed the practicality of cine sequence inpreoperative patient selection, syrinx analysis andpostoperative flow evaluation in anticipation of clinicaloutcome. Conclusion: Phase-contrast cine MRI is a useful tooldictated by resource availability. We recommend its routineuse in preoperative analysis and subsequent observationalfollow-up after surgery.

Modified atlantooccipital decompression combined with occipitocervical internal fixation in treatment of Chiari malformation type I with syringomyelia and atlantoaxial dislocation / 中华神经医学杂志

Objective:To investigate the clinical effect of modified atlantooccipital decompression combined with occipitocervical internal fixation on Chiari type I malformation combined with syringomyelia and atlantoaxial dislocation.Methods:Twenty-five patients with Chiari I malformation combined with syringomyelia and atlantoaxial dislocation accepted by modified atlantooccipital decompression combined with occipitocervical internal fixation in our hospital from January 2011 to March 2019. The clinical data of these patients were retrospectively analyzed. The changes of peak velocity of cerebrospinal fluid in the dorsal part of the spinal cord, electrophysiological results, atlantodental interval (ADI) values, sizes of syringomyelia, and Chicago Chiari outcome scale (CCOS) scores before and after operation were compared.Results:The peak velocity of cerebrospinal fluid in the dorsal spinal cord after surgery ([3.25±0.47] cm/s) was statistically higher in these patients than that before surgery ([2.13±0.19] cm/s, P<0.05). As compared with the preoperative results, ADI values, sizes of syringomyelia, and proportion of patients with abnormal electrophysiological monitoring at 6 months after surgery were significantly decreased, and CCOS scores at 6 months after surgery were significantly increased ( P<0.05). There were no new nerve function damage, infection, cerebrospinal fluid leakage, paralysis, respiratory failure or death. Conclusions:Modified atlantooccipital decompression combined with occipitocervical internal fixation plays effective role in atlantooccipital decompression and atlantoaxial anatomical reduction in patients with Chiari malformation type I combined with syringomyelia and atlantoaxial dislocation. The remission rate of syringomyelia is high, the symptoms and signs improve obviously, and the postoperative complications are less.

Factors predicting the occurrence of syringomyelia in patients with Chiari I malformations

Background: Chiari I malformations are defined as the downward herniation of cerebellar tonsils more than 5 mm through the foramen magnum. Syringomyelia is a common association of Chiari I malformation.Methods: This purpose of this study was to determine the various factors predicting the occurrence of syringomyelia in patients with Chiari I malformations. This retrospective study was conducted in Government medical college, Thrissur, Kerala, India. 27 patients admitted with diagnosis of symptomatic Chiari I malformations during the period of January 2015 to January 2019 were selected for the study.Results: The mean age was 22.8 years and syringomyelia was more common in patients older than 10 years (p value0.005). Females were 17(63%) and males were 10(37%) but there no difference in occurrence of syringomyelia among both sexes(p value is 0.16). Syringomyelia was present in 17(63%) cases. Association between various factors and syringomyelia measured by calculating P value which was 0.005 for age >10 years, 0.16 for sex, 0.093 for duration of symptoms >12 months, 0.05 for tonsillar descend >10mm and 0.097 for hypertension. The p value for the association of duration of symptoms >12months and extend of tonsillar descend is 0.001 Mean duration of symptoms (onset of first symptom to the time of presentation) was 16.3 months and syringomyelia was more common in patients with duration of symptoms more than 12 months but this difference was statistically not significant (p value 0.093). Mean tonsillar descend from the level of foramen magnum was 11.3 mms and syringomyelia was more common in patients with tonsillar herniation more than 10mm (p value 0.05). There is a statistically significant relationship between duration of symptoms more than 12 months and tonsillar descend more than 10mm(p value 0.001).There is no significant association between hypertension and occurrence of syringomyelia in patients with Chiari I malformation.Conclusions: Incidence of Chiari I Malformations is more among adults and it is slightly higher in females. Syringomyelia is a common association of Chiari I Malformations. The occurrence of syringomyelia in patients with Chiari I Malformations associated with increasing age of patients and extend of tonsillar herniation. There is no statistically significant association between syringomyelia and duration of symptoms or hypertension.