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La subluxación atlantoaxial es la lesión más frecuente en la columna cervical causada por la artritis reumatoidea. Se manifiesta por rigidez de nuca, dolor cervical y déficit neurológico. El diagnóstico se realiza con tomografía computarizada e imágenes de resonancia magnética. El intervalo atlanto dental anterior mayor a 5mm indica inestabilidad atlantoaxial, el intervalo atlanto dental posterior menor a 14mm advierte riesgo neurológico. Las indicaciones más frecuentes de cirugía son: dolor cervical severo, inestabilidad y síntomas de mielopatía. Cuando existe compresión medular es necesaria la descompresión cervical alta sea por vía posterior o por vía anterior (odontoidectomía endonasal versus transoral). La línea rinopalatina nos indicará la factibilidad de una odontoidectomía endonasal endoscópica (OEE). El objetivo de la presentación del presente caso es compartir nuestra experiencia con la primera odontoidectomía endonasal endoscópica realizada en nuestro país y fomentar la utilización de la técnica. La cirugía fue realizada en un paciente con cuadriparesia espástica por subluxación atlantoaxial por artritis reumatoidea y que presentó excelente evolución pos operatoria, con recuperación casi completa. La OEE es una técnica operatoria mínimamente invasiva, ideal para pacientes con múltiples comorbilidades y que ofrece de buenos a excelentes resultados.

Atlantoaxial subluxation is the most common injury to the cervical spine caused by rheumatoid arthritis. It is manifested by neck stiffness, neck pain and neurological deficit. Diagnosis is made with computed tomography and magnetic resonance imaging. The anterior dental atlanto interval greater than 5mm indicates atlantoaxial instability, the posterior dental atlanto interval less than 14mm warns of neurological risk. The most frequent indications for surgery are: severe neck pain, instability and symptoms of myelopathy. When there is spinal cord compression, upper cervical decompression is necessary, either via a posterior or anterior approach (endonasal versus transoral odontoidectomy). The rhinopalatine line will indicate the feasibility of an endoscopic endonasal odontoidectomy (EEO). The objective of the presentation of this case is to share our experience with the first endoscopic endonasal odontoidectomy performed in our country and to promote the use of the technique. The surgery was performed on a patient with spastic quadriparesis due to atlantoaxial subluxation due to rheumatoid arthritis and who presented excellent postoperative evolution, with almost complete recovery. EEO is a minimally invasive surgical technique, ideal for patients with multiple comorbidities and offering good to excellent results.

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Introducción: la subluxación atloaxoidea es un trastorno de la columna cervical, a nivel de las vértebras C1 y lC2 que causa deterioro en la rotación del cuello porque la faceta anterior de C1 se fija en la faceta de C2. Objetivo: enfatizar la importancia del diagnóstico temprano de la subluxación atlantoaxoidea en pacientes con o sin evidencia de trauma para que sea identificada como emergencia clínico quirúrgica. Desarrollo: en el presente trabajo se enfatiza en la necesidad de identificar eventos o complicaciones de la subluxación atloaxoidea que pongan en peligro la vida de los pacientes y requieran de la atención clínico quirúrgica de manera emergente por la compresión de médula espinal que puede ocasionar, de modo que en algunos reportes bibliográficos es clasificada como una emergencia. Conclusiones: existen muchas formas de presentación de la subluxación atloaxoidea, muchas de ellas pueden cursar con complicaciones que constituyan emergencias, así como variados tratamientos que deben ser valorados críticamente porque pueden ocasionar consecuencias mayores que la propia enfermedad, lo que habla a favor de lo imprescindible de un diagnóstico certero y de un enfoque multidisciplinar(AU)

Introduction: the atlantoaxial subluxation is a disorder of the cervical spine, at the level of the C1 and C2 vertebrae that causes deterioration in the rotation of the neck because the anterior facet of C1 is fixed on the facet of C2. Objective: To emphasize the importance of early diagnosis of atlantoaxial subluxation in patients with or without evidence of trauma to be identified as a surgical clinical emergency. Development: In the present work, emphasis is placed on the need to identify events or complications of atlantoaxial subluxation that endanger the life of patients and require surgical clinical attention in an emergent manner due to the compression of the spinal cord that may result from so that in some bibliographic reports it is classified as an emergency. Conclusions: There are many forms of presentation of atlantoaxial subluxation, many of them can present complications that constitute emergencies, as well as various treatments that must be critically evaluated because they can cause greater consequences than the disease itself, which speaks in favor of the essential of an accurate diagnosis and a multidisciplinary approach(AU)

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In the present article, we describe a technique of direct intraoperative reduction of congenital atlantoaxial subluxation associated with severe basilar invagination and atlas assimilation. It consists of a wide dissection of the craniovertebral junction, exposing the region between the superior facet of C2 and the occiput (described as a "pseudoarticulation" by some authors). After exposure, a self-rotating small dissector or Kobe is used bilaterally, pushing C2 anteriorly and inferiorly, with reduction of the dislocation, followed by craniovertebral fixation. The technique is safe and eliminates the need for an anterior approach to the odontoid reduction, as well as the need to dissect the C1­2 joint.

Neste artigo, descrevemos a técnica de redução intraoperatória direta de subluxação atlantoaxial congênita associada a invaginação basilar grave e assimilação do atlas. Ela consiste em ampla dissecção da junção craniovertebral, expondo a região entre a face superior da C2 e o osso occipital (descrita como uma "pseudoarticulação" por alguns autores). Após exposição, um descolador pequeno ou Kobe é usado bilateralmente com rotação própria, deslocando C2 anterior e inferiormente, com redução da luxação, seguido de fixação craniovertebral. A técnica é segura e elimina a necessidade de uma abordagem anterior para a ressecção do odontoide, além de evitar a dissecação da junta C1­C2.

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BACKGROUND CONTEXT: Many etiologies can lead to atlantoaxial subluxaion. In Grisel syndrome (GS), this subluxation occurs spontaneously after inflammatory processes of the head and neck. Diagnosis is typically based on clinical history and a strong suspicion of this syndrome. Nonsurgical treatment most often resolves the symptoms; however, in some cases surgical treatment is necessary to repair the subluxation. Various surgical techniques and instrumentation systems have been used to treat atlantoaxial subluxation, although there is no consensus regarding the best treatment method for the pediatric population. PURPOSE: To describe a case of atlantoaxial subluxation in a child with GS treated surgically with an alternative construct. STUDY DESIGN/SETTING: This is a case report and literature review. MATERIALS AND METHODS: Our case study involves a 5-year-old girl with a 6-month history of unresolved Fielding type II atlantoaxial subluxation caused by GS. Despite conservative treatment, the patient's symptoms continued to progress. After two failed closed reduction attempts, open reduction and C1-C2 fusion were performed with atlas laminar hook and axis pedicle polyaxial screws. A literature review of the surgical treatment of GS was also performed. RESULTS: After surgery, the patient exhibited full clinical and functional recovery with complete resolution of symptoms. At the 36-month follow-up examination, there was continual evidence of satisfactory reduction and fusion. No complications were observed. Upon completion of the literature review, eight GS cases were found to have been treated surgically with the minimum patient age being 9 years. CONCLUSIONS: Conservative management of GS is the most common and effective treatment; however, a few surgical cases have been reported in the literature with good results. Satisfactory clinical results and fusion at 36 months post surgery were seen in a pediatric patient with atlantoaxial subluxation and instability using atlas laminar hook and axis pedicle polyaxial screws.

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A instabilidade da articulação atlantoaxial é uma afecção comum entre cães de raça de pequeno porte, e os sinais clínicos desenvolvem-se com menos de dois anos de idade, geralmente são progressivos e o paciente apresenta hiperestesia cervical, ataxia proprioceptiva, e em casos severos paraplegia. A instabilidade pode gerar subluxação dorsal do axis em relação ao atlas, levando a uma lesão medular em diferentes níveis. Alterações congênitas, como ausência ou hipoplasia do processo odontóide e ligamentos, ou traumáticas, como fraturas do processo odontóide e ruptura dos ligamentos estão envolvidas na fisiopatologia da afecção. O diagnóstico pode ser realizado com auxílio de radiografias lateral e ventrodorsal com a região craniocervical em posição neutra. O tratamento clínico é a escolha primária e faz-se o uso de colar cervical por três meses, confinamento e corticosteróides. Exceção a este tratamento são pacientes com apresentação clínica severa ou refratários ao tratamento conservativo, nestes casos indica-se a cirurgia para estabilização por meio de abordagens ventrais ou dorsais. Observou-se que as técnicas cirúrgicas ventrais apresentam maiores índices de sucesso e menores recorrências de recidivas, contudo, as estabilizações cirúrgicas continuam a ser um desafio pela elevada taxa de complicações e óbitos transoperatórios.

The instability of the atlantoaxial joint is a common condition among dogs small breed, and clinical signs are developed under two years of age, are usually progressive and the patient presents cervical hyperesthesia, ataxia proprioceptive, and in severe cases tetraplegia. The instability can generate subluxation dorsal of axis in relation to the atlas, leading to spinal cord injury at different levels. Congenital abnormalities, such as absence or hypoplasia of the odontoid process and ligaments, or traumatic, such as fractures of the odontoid process and rupture of ligaments are involved in the pathophysiology of the disease. The diagnosis can be performed with the aid of lateral X-rays with the DV region craniocervical in neutral position. Clinical treatment is the primary choice and makes the use of cervical collar for three months confinement and corticosteroids. Exception to this treatment are patients with severe clinical presentation or refractory to conservative treatment in these cases is indicated surgery to stabilize through the ventral or dorsal approaches. It was observed that the ventral surgical techniques have higher success rates and lower recurrence of relapse, however, surgical stabilization remains a challenge due to high rate of intraoperative complications and death.

La inestabilidad de la articulación atlantoaxial es una condición común entre los perros de raza pequeña, y los signos clínicos se desarrollan bajo dos años de edad, son por lo general progresiva y el paciente hiperestesia cervical, ataxia propioceptiva, y en casos severos tetraplejia. La inestabilidad puede generar eje dorsal subluxación en relación con el atlas, lo que lleva a una lesión de la médula espinal en los diferentes niveles. Anomalías congénitas, tales como ausencia o hipoplasia de la apófisis odontoides y ligamentos, o traumática, tales como fracturas de la apófisis odontoides y la rotura de los ligamentos están implicados en la fisiopatología de la enfermedad. El diagnóstico se puede realizar con la ayuda de los rayos X en la región lateral VD craneocervical en posición de punto muerto. El tratamiento clínico es la elección primaria y hace que el uso del collarín cervical durante tres meses de confinamiento y corticosteroides. Excepción a este tratamiento son los pacientes con presentación clínica grave o refractario al tratamiento conservador en estos casos la cirugía está indicada para estabilizar a través de los enfoques ventral o dorsal. Se observó que las técnicas quirúrgicas ventrales tienen mayores tasas de éxito y menor recurrencia de recaídas, sin embargo, la estabilización quirúrgica sigue siendo un desafío debido a la alta tasa de complicaciones intraoperatorias y muertes.

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OBJECT: The goal of this study was to compare the indications, benefits, and complications between the endoscopic endonasal approach (EEA) and the microscopic transoral approach to perform an odontoidectomy. Transoral approaches have been standard for odontoidectomy procedures; however, the potential benefits of the EEA might be demonstrated to be a more innocuous technique. The authors present their experience with 12 consecutive cases that required odontoidectomy and posterior instrumentation. METHODS: Twelve consecutive cases of craniovertebral junction instability with or without basilar invagination were diagnosed at the National Institute of Neurology and Neurosurgery in Mexico City, Mexico, between January 2009 and January 2013. The EEA was used for 5 cases in which the odontoid process was above the nasopalatine line, and was compared with 7 cases in which the odontoid process was beneath the nasopalatine line; these were treated using the transoral microscopic approach (TMA). Odontoidectomy was performed after occipital-cervical or cervical posterior augmentation with lateral mass and translaminar screws. One case was previously fused (Oc-C4 fusion). The senior author performed all surgeries. American Spinal Injury Association scores were documented before surgical treatment and after at least 6 months of follow-up. RESULTS: Neurological improvement after odontoidectomy was similar for both groups. From the transoral group, 2 patients had postoperative dysphonia, 1 patient presented with dysphagia, and 1 patient had intraoperative CSF leakage. The endoscopic procedure required longer surgical time, less time to extubation and oral feeding, a shorter hospital stay, and no complications in this series. CONCLUSIONS: Endoscopic endonasal odontoidectomy is a feasible, safe, and well-tolerated procedure. In this small series there was no difference in the outcome between the EEA and the TMA; however, fewer complications were documented with the endonasal technique.

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Mucopolysaccharidosis type IVA is a rare lysosomal storage disease caused by a deficiency of N-acetylgalactosamine 6-sulfatase. Studies usually focus on skeletal abnormalities and their consequences. This study explores the neurological manifestations in a cohort of mucopolysaccharidosis type IVA patients, with a detailed focus on brain and spinal magnetic resonance imaging (MRI) findings. We performed a cross-sectional study involving nine patients with a biochemical confirmation of mucopolysaccharidosis type IVA. The protocol consists of a comprehensive clinical examination and brain and spinal cord MRI analysis for all subjects. The mean age was 16.4 years (±5.7) and the mean onset of symptoms was 11.5 months (±6.3). Overall, cognition was spared in all but one patient and motor weakness was a constant finding in all patients. Deep sensation impairment was found in six patients. The brain MRIs showed non-specific white matter changes in two patients. Other abnormalities such as clival hypoplasia, basilar invagination, and arachnoid cists appeared in seven of the nine patients. Eight patients presented spinal cord compression, and in three of them, two spinal levels were compromised. Odontoid hypoplasia and degenerative features in the neuroaxis were present in all patients. Our experience with mucopolysaccharidosis type IVA patients supports the evidence of central nervous system involvement. We emphasize the importance of regular clinical assessments with complete MRI studies, as an attempt to detect the early signs of spinal cord compression. This evaluation may be especially important before surgical interventions, as occult lesions may become symptomatic and promote postoperative unfavorable outcomes.

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PURPOSE: The objective of this paper is to present a case series of patients with diagnosis of torticollis due to Grisel's syndrome and, in doing so, raise awareness of an unusual condition that could be fatal. A review of the literature is presented regarding diagnosis and treatment. METHODS: Case series of three patients that were 7, 10, and 12 years old with history of tonsillitis or neck surgery consulted to the service of Rehabilitation Medicine. RESULTS: Physical examination showed a fixed head or limitation of movement to neutral position and initially a normal neurological examination. Initial cervical X-rays were not diagnostic. The final diagnosis was made by CT scan or MRI. All 3 patients were treated with anti-inflammatory medications, immobilization, and/or cervical traction or surgery. CONCLUSION: Grisel's syndrome is a non-traumatic atlanto-axial rotatory fixation (AARF) with or without subluxation following infection or surgery in the head or neck region. This paper presents an unusual cause of torticollis that could be fatal or cause neurological injury if not recognized and treated appropriately.

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Objetivo. Describir un caso de subluxación atlantoaxial anterior secundaria a una osteoartritis. Descripción. Un paciente masculino de 60 años de edad con una tetraparesia moderada, rápidamente progresiva, piramidalismo y trastornos sensitivos superficiales y profundos, de cuatro meses de evolución. Las radiografías de columna cervical frente y perfil muestraron una subluxación atlanto-axial anterior. La resonancia magnética mostró un tejido patológico periodontoideo con extensión a articulares y mielomalacia compresiva a nivel C1-C2 a expensas de elementos posteriores por la subluxación. Las radiografías del resto de la columna, manos, rodillas y hombros mostraron cambios degenerativos artrósicos. Se realiza una interconsulta con el servicio de reumatología que, luego del analizar los resultados serológicos, clínicos y radiológicos, arribó al diagnóstico de osteoartritis. Intervención. Consistió en un abordaje posterior, con apertura del foramen magno, laminectomía de C1 y osteosíntesis occipitocervical con la colocación de injertos óseos autólogos. Conclusión. La osteoartritis es una causa infrecuente de subluxación atlantoaxial anterior. Más allá de la patología causal, sabemos que el tratamiento quirúrgico es fundamental en los pacientes sintomáticos.

Objetive. To describe a new case of an anterior atlanto-axial suluxation caused by osteoarthritis. Dewscription. A 60-year-old male patient presented a 4 month history of progressive cuadriparesis, bipyramidalism and deep and superficial hipoestesia. The anterior and lateral radiographs showed an anterior C1-C2 subluxation and the magnetic resonance periodontoidal tissue mass that caused a compressive myelopathy. Intervention. Through a posterior approach, we openned theforamen magnum and performed a C1 laminectomy. After the decompression we performed an occipitocervical arthrodesis with an "Y" plate implant and autologous bone grafts. Conclusion. Osteoarthritis is an unusual cause of anterior atlantoaxial subluxation, that must be treated surgically in symptomatic patients.

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Neste estudo foram analisadas ficha clínica e radiografias simples de cães com diagnóstico de subluxação atlantoaxial congênita, obtidos junto ao Serviço de Radiologia do Hospital Veterinário da Faculdade de Medicina Veterinária e Zootecnia da Universidade de São Paulo, no período de janeiro de 1990 a dezembro de 1999. Durante este período, foram identificados sete cães com alterações do processo odontóide do áxis (agenesia ou hipoplasia), levando à instabilidade da articulação atlantoaxial e conseqüente compressão medular. Todos os animais, com idade igual ou inferior a um ano, apresentavam sinais neurológicos. O diagnóstico definitivo foi firmado mediante as alterações observadas em exame radiográfico simples.(AU)

This retrospective study reviews the clinical signs and plain radiographic findings in dogs with congenital atlantoaxial subluxation. The data were obtained from the Radiology Department of the Veterinary Teaching Hospital, School Veterinary Medicine of University of São Paulo during period from january 1990 to december 1999. In this 10-year period, seven dogs were reported with malformation of dens axis (agenesis or hypoplasia), what caused articular instability and spinal cord compression. All the animals were one year old or younger and all had neurologic signs. Diagnosis was made with plain cervical radiographs. (AU)

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