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)

Dorsal arachnoid web: A rare cause of syringomyelia and myelopathy.

Dorsal arachnoidal webs are condensations of arachnoidal tissue densely adherent to the pial surface of the posterior aspect of the dorsal spinal cord. Infrequently described in literature they disrupt the flow of CSF in the spinal subarachnoid space leading to syringomyelia and myelopathy. While there are several theories on their origin, the "scalpel sign" on magnetic resonance imaging is considered to be pathognomonic of this condition. An illustrative case of a 58 year old man with syringomyelia and dorsal cord indentation who presented with spastic paraparesis, gait instability, parasthesias and bilateral non radicular upper limb pain that resolved following excision of the web is described to highlight the importance of considering this diagnosis when cases of so called "idiopathic" syringomyelia are encountered.

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.

Myelopexy: A novel technique in posttraumatic syringomyelia.

Posttraumatic syringomyelia (PTS) is a well-reported phenomenon that usually takes place in the long-term course of patients following spinal cord injury. Different surgical procedures have been described: spinal cordectomy is usually a last option technique, but might be an excellent choice in patients with severe spinal cord injuries. We present a young patient with complete spinal cord injury after spine trauma, who developed posttraumatic syringomyelia with progressive motor deterioration twelve years after fixation. We performed a novel surgical technique (myelopexy) with excellent resolution of syringomyelia, sparing the negative implications of complete cord transection. Some artistic illustrations made by one of the corresponding authors are included, to better understanding of operative details.

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.

Myelopexy: A novel technique in posttraumatic syringomyelia.

Posttraumatic syringomyelia (PTS) is a well-reported phenomenon that usually takes place in the long-term course of patients following spinal cord injury. Different surgical procedures have been described: spinal cordectomy is usually a last option technique, but might be an excellent choice in patients with severe spinal cord injuries. We present a young patient with complete spinal cord injury after spine trauma, who developed posttraumatic syringomyelia with progressive motor deterioration twelve years after fixation. We performed a novel surgical technique (myelopexy) with excellent resolution of syringomyelia, sparing the negative implications of complete cord transection. Some artistic illustrations made by one of the corresponding authors are included, to better understanding of operative details.

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.

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

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

Arnold Chiari tipo I y siringomielia en el adulto / Arnold Chiari type I and siringomyelia in the adult: report of a case

La malformación de Arnold Chiari es una anomalía congénita caracterizadapor el defecto de la base del cráneo y la herniación de parte del tronco y cerebelo a través del agujero magno, y puede estar asociado a siringomielia o hidrocefalia, la prevalencia de la enfermedad oscila entre el 4.3 x 1/100000 habitantes, afecta a ambos sexos, con ligero predominio en las mujeres, se trata de una paciente de 58 años de edad con antecedentes patológicos de relevancia, psoriasis del cuero cabelludo hace 8 años, gastritis y alergia a la aspirina y sus derivados, que fue hospitalizado porcefalea, debilidad muscular progresiva que inicia en miembro inferior izquierdo luego a miembros superiores bilateralmente, problemas del equilibrio, dolor en el cuello los brazos y la espalda, los estudios diagnósticos por imagen de resonancia magnética (RMN) de cerebro y columna cervico-dorsal ha demostrado una herniación de más 5mm por debajo del foramen magno. La siringomielia con malformación de Arnold Chiari tipo I es rara en adultos.

The Arnold Chiari malformation is a congenital anomaly characterized by the defect of the base of the skull and the herniation of part of the trunk and cerebellum through the foramen magnum, and may be associated with syringomyelia or hydrocephalus, the prevalence of the disease ranges from 4.3 x 1/100000 inhabitants, affects both sexes, with a slight predominance in women, this is a 58-year-old patient with relevant pathological history, psoriasis of the scalp 8 years ago, gastritis and allergy to aspirin and its derivatives, who was hospitalized for progressive muscle weakness headache that starts in the left lower limb after upper limbs bilaterally, balance problems, pain in the neck, arms and back, diagnostic studies by magnetic resonance imaging (MRI) of the brain and cervico-dorsal spine has shown a herniation of more than 5mm below the foramen magnum. Syringomyelia with Arnold Chiari type I malformation is rare in adults.

Pain treatment and recovery of functionality in a former athlete diagnosed with myofascial pain syndrome in the course of syringomyelia. Case report / Tratamento da dor e recuperação da funcionalidade em ex-atleta diagnosticada com síndrome dolorosa miofascial no curso de siringomielia. Relato de caso

ABSTRACT BACKGROUND AND OBJECTIVES: There are few studies that address non-surgical treatment in cases of syringomyelia, which reduces the possibilities of treatment for the patient. The objective of this study was to analyze the efficacy of the physiotherapeutic treatment for the symptoms of the pathology. CASE REPORT: Idiopathic syringomyelia is a condition in which a cystic-shaped cavity appears within the spinal cord. After the diagnosis of syringomyelia in C3-C6, 3 years ago, the patient, a former volleyball athlete, remained stable with no anesthetic dissociation, muscle atrophy or limb paresthesia. However, she began to have constant back and neck pain to the extent of limiting her functioning in jogging, volleyball, and difficulties of movement in daily routine. The physiotherapeutic treatment in 6 sessions, worked in the muscle and fascial release with dry needling and manual myofascial release associated with specific vertebral adjustments with chiropractic techniques, and after the cessation of the pain, specific muscle strengthening exercises. CONCLUSION: Physiotherapy showed to be an effective treatment for patient with syringomyelia that presented symptoms to myofascial pain syndrome.

RESUMO JUSTIFICATIVA E OBJETIVOS: Existem poucos estudos que abordam o tratamento não cirúrgico nos casos de siringomielia, fato que diminui as possibilidades de tratamento para o paciente. O objetivo deste estudo foi analisar a eficácia do tratamento fisioterapêutico para os sintomas da doença. RELATO DO CASO: A siringomielia idiopática é uma doença na qual uma cavidade em forma de cisto aparece dentro da medula espinhal. Após o diagnóstico de siringomielia em C3-C6, 3 anos atrás, a paciente, ex-atleta de voleibol, se manteve com o quadro estável e sem dissociação anestésica, atrofia muscular ou parestesia de membros, porém começou a ter dores lombares e cervicais constantes a ponto de ter limitação da função em corrida, voleibol e dificuldades de movimentação no dia a dia. Durante o tratamento fisioterapêutico em 6 sessões foi abordada a liberação muscular e fascial a partir do agulhamento à seco e liberação miofascial manual associado a ajustes vertebrais específicos com técnicas quiropráticas, e após cessamento das dores fortalecimento muscular específico. CONCLUSÃO: A Fisioterapia demonstrou ser um tratamento eficaz em uma paciente com siringomielia que apresentava sintomas relacionados à síndrome dolorosa miofascial.

Un enfoque clínico e histórico de la Malformación de Chiari Tipo I en adultos / A clinical and historic approach Type I Chiari malformation in adults

RESUMEN Se revisó la literatura existente sobre la Malformación de Chiari Tipo I en el adulto, haciendo énfasis en su diagnóstico imagenológico y tratamiento médico-quirúrgico. La malformación de Chiari es una enfermedad poco frecuente. Representa entre el 1 y el 4 % de todas las patologías neuroquirúrgicas. El diagnóstico se realiza meses y hasta años después de comenzada la sintomatología y se confirma en el 100 % de los casos con resonancia magnética de cráneo. El tratamiento es quirúrgico en los pacientes sintomáticos, siendo controversial en aquellos oligosintomáticos o con diagnóstico casual. La cirugía siempre debe realizarse con el apoyo de monitoreo neurofisiológico, que puede ser determinante en la técnica quirúrgica a emplear. La mortalidad asociada a la cirugía es muy baja, oscilando entre el 0 y 0,5 % según la mayoría de las series. Lo más importante es la selección de los pacientes para el tratamiento quirúrgico. No debe asumirse una actitud expectante en espera de un deterioro neurológico que justifique la cirugía, cuando ésta se hace a tiempo los resultados son mejores y con un mínimo de complicaciones (AU).

ABSTRACT The existent literature on Type I Chiari Malformation in adults was reviewed, making emphasis in its imaging diagnosis and medico-surgical treatment. Chiari malformation is a few frequent disease. It represents between 1 % and 4 % of all the neurosurgery pathologies. The diagnosis is made up months and even years after the beginning of the symptoms and it is confirmed by cranial magnetic resonance in the 100 % of the cases. The treatment is surgical in symptomatic patients, and it is controversial in the oligosymptomatic ones and in those with casual diagnosis. The surgery should be performed with the support of neurophysiological monitoring that might be determinant in the surgical technique to use. The mortality associated to the surgery is low, ranging from 0 and 0.5 % in most of the series. The most important thing is the choice of the patients for the surgical treatment. The neurological deterioration should not be expected to justify the surgery; when the surgery is carried out on time the results are better and with the minimum of complications (AU).

Pan, Syrinx and syringomyelia / Pan, Syrinx e siringomielia

ABSTRACT Many myths and legends have had a deep influence on modern language, and on modern medical vernacular. The terms "syrinx" and "panic"are two of the most characteristic examples and their use in neurology and other specialties is well known. This article reviews the history of these words in Greek mythology and their use in modern medicine. It is known by very few that clinical symptoms or conditions, such as syringomyelia and panic attacks, have a mythological origin in their definition and naming.

RESUMO Muchos mitos y leyendas han tenido una profunda influencia sobre el lenguaje y el uso del argot médico moderno. Los términos "syrinx" y "pánico" son dos de los ejemplos más característicos y su uso en neurología y otras especialidades es bien conocido. Este artículo revisa la historia de estas palabras en la mitología griega y su uso en la medicina moderna. Es conocimiento de pocos que síntomas o condiciones como la siringomielia o los ataques de pánico, tienen un origen mitológico en su definición y denominación.

Malformación de Chiari Tipo I en adultos. Un enfoque médico-quirúrgico. Parte I / Type I Chiari malformation in adults. A medico-surgical approach. Part I

Se revisó la literatura existente sobre la Malformación de Chiari Tipo I en el adulto, haciendo énfasis en su diagnóstico imagenológico y tratamiento médico-quirúrgico. La malformación de Chiari es una enfermedad poco frecuente. Representa entre el 1 y el 4% de todas las patologías neuroquirúrgicas. El diagnóstico se realiza meses y hasta años después de comenzada la sintomatología y se confirma en el 100% de los casos con Resonancia magnética de cráneo. El tratamiento es quirúrgico en los pacientes sintomáticos, siendo controversial en aquellos oligosintomáticos o con diagnóstico casual. La cirugía siempre debe realizarse con el apoyo de monitoreo neurofisiológico, que puede ser determinante en la técnica quirúrgica a emplear. La mortalidad asociada a la cirugía es muy baja, oscilando entre el 0 y 0,5% según la mayoría de las series. Lo más importante es la selec­ción de los pacientes para el tratamiento quirúrgico. No debe asumirse una actitud expectante en espera de un deterioro neurológico que justifique la cirugía, cuando ésta se hace a tiempo los resultados son mejores y con un mínimo de complicaciones (AU).

The aim of this article is reviewing the existent literature on Type I Chiari malformation in adults and making emphasis in its imaging diagnosis and medico-surgical treatment. Chiari malformation is a few frequent diseases. It represents 1-4 % of all the neurosurgical diseases. The diagnosis is made months and even years after the beginning of the symptoms and it is confirmed by cranial magnetic resonance in the 100 % of the cases. The treatment is surgical in symptomatic patients, and it is controversial in the oligosymptomatic ones and in those with casual diagnosis. The surgery should be performed with the support of neurophysiological monitoring that might be determinant in the surgical technique to use. The mortality associated to the surgery is low, ranging from 0 and 0.5 % according to most of the series. The most important thing is the choice of the patients for the surgical treatment. The neurological deterioration should not be expected to justify the surgery; when the surgery is carried out on time the results are better and with the minimum of complications (AU).

Thecaloscopy: a novel method in spine surgery / Tecaloscopia: um novo método na cirurgia de coluna / Tecaloscopia: un nuevo método en la cirugía de la columna vertebral

ABSTRACT Objetive: Thecaloscopy is a less invasive method of exploration of the spinal subarachnoid space, using an ultra-thin, flexible endoscope and endoscopic fenestration of scars and adhesions. Thecalopscopy was used in Russian neurosurgery for the first time. Methods: Since 2009, we have operated on 32 patients with the following diagnoses: 17 - spinal adhesive arachnoiditis (8 - local forms, 9 - diffuse forms), 12 - spinal arachnoid cysts (7 - post-traumatic cysts, 5 - idiopathic cysts), and 3 - extramedullary tumors (thecaloscopic videoassistance and biopsy). In all cases, we performed exploration of subarachnoid space and pathologic lesion with endoscopic perforation of the cyst or dissection of adhesions using special instrumentation. The mean follow-up time in our group was 11.4 months. Results: Neurological improvement (mean 1.4 by the modified Frankel scale, 1.8 by the Ashworth spasticity scale) was seen in 87% of patients operated for spinal arachnopathies. Temporary neurological deterioration (mild disturbances of deep sensitivity) was seen in 9% of patients and managed successfully with conservative treatment. One patient (3.1%) was operated three times due to relapse of adhesions. There were no serious intraoperative complications (e.g. severe bleeding or dura perforation). Postoperative complications included one CSF leakage and one case of postoperative neuralgic pain. The mean hospitalization time was 7.6 days. Conclusion: According to our data, we conclude that thecaloscopy is efficient and safe method, and should be widely used for spinal arachnopathies, adhesive arachnoiditis and arachnoid cysts. Taking into account that adhesive spinal arachnoiditis is a systemic process, and that spinal arachnoid cysts may also be extended, thecaloscopy may be regarded as the most radical and less-invasive form of surgical treatment that currently exists in neurosurgery.

RESUMO Objetivo: A tecaloscopia é um método menos invasivo de exploração do espaço subaracnóideo, com um endoscópio flexível ultrafino e fenestração endoscópica de cicatrizes e aderências. A tecaloscopia foi usada pela primeira vez na neurocirurgia russa. Métodos: Desde 2009, operamos 32 pacientes com os seguintes diagnósticos: 17 aracnoidites adesivas espinais (8 formas locais e 9 formas difusas), 12 cistos aracnóideos espinais (7 cistos pós-traumáticos e 5 cistos idiopáticos) e 3 tumores extramedulares (vídeo-assistida por tecaloscopia e biópsia). Em todos os casos, realizamos a exploração do espaço subaracnóideo e da lesão com perfuração endoscópica do cisto ou dissecção de aderências usando instrumentação especial. O acompanhamento médio em nosso grupo foi de 11,4 meses. Resultados: A melhora neurológica (média 1,4 pela escala de Frankel modificada e 1,8 pela escala de espasticidade de Ashworth) foi observada em 87% dos pacientes operados devido a aracnopatias da coluna vertebral. A deterioração neurológica temporária (distúrbios leves da sensibilidade profunda) foi observada em 9% dos pacientes que foi tratada com sucesso pelo método conservador. Um paciente (3,1%) foi operado três vezes devido à recorrência de aderências. Não houve complicações intraoperatórias graves (por exemplo, sangramento grave ou perfuração da dura-máter, etc.). As complicações pós-operatórias incluíram um extravasamento de LCE e um caso de neuralgia pós-operatória. O período médio de hospitalização foi de 7,6 dias. Conclusão : De acordo com nossos dados, concluímos que a tecaloscopia é um método eficiente e seguro, e deve ser amplamente utilizado para aracnopatias espinais, aracnoidites adesivas e cistos aracnóideos. Considerando que a aracnoidite adesiva é um processo sistêmico e que os cistos aracnóideos da coluna vertebral também podem ser extensos, a tecaloscopia pode ser considerada como a forma mais radical e menos invasiva de tratamento cirúrgico existente atualmente em neurocirurgia.

RESUMEN Objetivo: La tecaloscopia es un método de exploración menos invasivo del espacio subaracnoideo con un endoscopio flexible ultrafino y fenestración endoscópica de cicatrices y adherencias. La tecaloscopia se utilizó en neurocirugía rusa por primera vez. Métodos: Desde 2009 operamos 32 pacientes con los siguientes diagnósticos: 17 aracnoiditis adhesivas espinales (8 formas locales y 9 formas difusas), 12 quistes aracnoideos espinales (7 quistes postraumáticos y 5 quistes idiopáticos) y 3 tumores extramedulares (asistida por video tecaloscópico y biopsia). En todos los casos se realizó la exploración del espacio subaracnoideo y lesión con perforación endoscópica del quiste o disección de adherencias mediante instrumentación especial. El seguimiento medio en nuestro grupo fue de 11,4 meses. Resultados: La mejoría neurológica (media 1,4 por la escala de Frankel modificada y 1,8 por la escala de espasticidad de Ashworth) se observó en el 87% de los pacientes operados debido a aracnopatías de la columna vertebral. Se observó deterioro neurológico temporal (alteraciones leves de la sensibilidad profunda) en el 9% de los pacientes, que fue tratada con éxito por el método conservador. Un paciente (3,1%) fue operado tres veces debido a la recurrencia de las adherencias. No hubo complicaciones intraoperatorias graves (por ejemplo, sangrado grave o perforación de la duramadre, etc.). Las complicaciones postoperatorias incluyeron una extravasación del LCR y un caso de neuralgia postoperatoria. El período promedio de hospitalización fue de 7,6 días. Conclusiones : De acuerdo con nuestros datos, concluimos que la tecaloscopia es un método eficiente y seguro, que debe ser ampliamente utilizado para aracnopatías espinales, aracnoiditis adhesivas y quistes aracnoideos. Teniendo en cuenta que la aracnoiditis adhesiva es un proceso sistémico y que los quistes aracnoideos de la columna vertebral también pueden ser extensos, la tecaloscopia puede ser considerada como la forma más radical y menos invasiva de tratamiento quirúrgico existente actualmente en neurocirugía.

Evolución Natural De La Siringomielia: Presentación De Un Caso / Natural Evolution Of Syringomyelia: Case Report

RESUMEN La siringomielia es una enfermedad crónica degenerativa y progresiva de la médula espinal que se caracteriza por una pérdida disociada de la sensibilidad y amiotrofia braquial, con evidencia de cavitación centromedular. Presentamos el caso de una paciente de 62 años de edad, con siringomielia asociada a malformación de Chiari tipo 1 que rechazó espontáneamente cualquier intervención quirúrgica a los cinco años de su diagnóstico por lo cual se ha realizado un seguimiento de 21 años de evolución. Las imágenes de resonancia magnética demuestran la evolución natural de la siringomielia desde 1998 hasta la actualidad.

SUMMARY Syringomyelia is a chronic degenerative and progressive disease of the spinal cord characterized by a dissociated loss of brachial sensitivity and amyotrophy, with evidence of centromedullary cavitation. We report the case of a 62-year-old female patient with syringomyelia associated with Arnold Chiari malformation (type 1) who spontaneously rejected any surgical intervention five years after her diagnosis, which has been followed up for 21 years. Magnetic resonance imaging demonstrates the natural evolution of syringomyelia from 1998 to the present.

Treatment of Craniodiaphyseal Dysplasia Presenting with Chiari Type-I

Objective Chiari malformation type-I (CM-1) is described radiographically as a simple displacement of the cerebellar tonsils at least 5 mm below the foramen magnum (FM). If CM-1 exists due to hyperostosis of the cranial bones, the authors were not able to determine a common consensus for the treatment of CM-1 and syringomyelia. Methods A 31-year-old-female presented to our hospital with bilateral facial paralysis, hypoesthesia and motor loss of the extremities. The patient had bilateral gag reflex loss, phonation disorder and dysarthric speaking. Sensory and motor deficits were available at the bilateral upper and lower extremities. The skeletal radiographs revealed extensive thickening and sclerosis of the calvarial and facial bones, moderate widening and sclerosis of the clavicles and ribs, and that the internal auditory canal (IAC) and the optic foramen (OF) were narrowed. CM-1 and syringomyelia secondary to the small posterior fossa were due to calvarial hyperostosis. The patient underwent posterior fossa decompression and duraplasty. In addition, a syringosubarachnoid shunt was placed at the level of C7-T1. The symptoms of lower cranial nerve palsy and motor loss were recovered, but the symptoms of the foraminal stenosis, such as visual and auditory losses and facial paralysis were not recovered in any way. Conclusion We described in this case report CM-1 as a late complication of craniodiaphyseal dysplasia (CDD), and the difficulties in its treatment. In the treatment of these patients with CDD, posterior fossa decompression and syringosubarachnoid shunting are necessary, in spite of all the risks of these procedures.

Objetivo A malformação Chiari tipo-I (MC-1) é descrita radiograficamente como um simples deslocamento da tonsila cerebelar de pelo menos 5 mm abaixo do forame magno (FM). Se houve MC-1 em função da hiperostose dos ossos do crânio, os autores não foram capazes de determinar um consenso para o tratamento de MC-1 e siringomielia. Métodos Paciente de 31 anos deu entrada com paralisia facial bilateral, hipoestesia e perda motora das extremidades. A paciente apresentou perda de reflexo de vômito bilateral, desordem fonética e disartria. Deficiência motora e sensorial foram identificadas em ambas as extremidades superiores e inferiores. Radiografias do esqueleto revelaram extensa espessamento e esclerose dos ossos calvários e faciais, ampliação moderada e esclerose das clavículas e costelas, além de estreitamento do canal auditivo interno (CAI) e do forame ótico (FO). A MC-1 e siringomielia secundária à pequena fossa posterior ocorreram devido à hiperosteose calvarial. A paciente foi submetida a descompressão da fossa posterior e duroplastia. Somado a isso, um shuntsiringo-subaracnóideo foi colocado na altura da C7-T1. Os sintomas de paralisia donervo craniano inferior e a perda motora foram recuperados, mas os sintomas de estenose do forame, como por exemplo perdas auditivas e visuais, ou paralisia facial, não foram recuperados em nenhum nível. Conclusão descrevemos neste relato de caso a MC-1 como uma complicação posterior da displasia craniodiafisária (DCD), além das dificuldades em seu tratamento. No tratamento desta paciente com DCD, são necessários a descompressão da fossa posterior e o shunt siringo-subaracnóideo, apesar de todos os riscos neste procedimento.