Research Progress on the Application of Goel Technique in Craniovertebral Junction Anomalies / 中国医学科学院学报

Craniovertebral junction anomalies are a group of diseases characterized by the pathological changes of occipital bone,atlantoaxial bone,cerebellar tonsil,surrounding soft tissue,and nervous system,which are caused by a variety of factors.Chiari malformation is a common type of craniovertebral junction anomalies,the conventional surgical therapy of which is posterior fossa decompression.Currently,scholars represented by Goel have proposed a new theory on the classification,pathogenesis,and treatment of Chiari malformation based on posterior atlantoaxial fixation (Goel technique).This article introduces the progress in Goel technique,aiming to provide reference for the clinical work.

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.

First occurrence of Arnold Chiari type II malformation and associated abnormalities in a Gir calf produced in vitro from Brazil - case report / [Primeira ocorrência de malformação de Arnold Chiari tipo II e anormalidades associadas em uma bezerra Gir de produção in vitro no Brasil - relato de caso]

This study characterized the clinical, radiological, ultrasound, and necroscopic findings of a case of Arnold-Chiari type II malformation in a Gir breed calf from Brazil. The animal was hospitalized at sixty days of age, in permanent sternal recumbency, cutaneous appendix at the 4th lumbar vertebra and kyphoscoliosis of the caudal and lumbosacral thoracic spine. Radiographic examination of the spine and skull revealed spina bifida and suspected occipital hypoplasia. Upon examination of myelography with an injection of lumbar and atlantooccipital contrast, it was possible to visualize the meningocele at the 4th lumbar vertebra region and findings at the rhombencephalon level of increased regional pressure with failure to fill the contrast in the posterior fossa, in the presence of clear demarcation of the circumvolutions of the cerebral cortex and the subarachnoid space of the cervical spinal cord. Ultrasonographic examination of the cerebellum showed an insinuation of the cerebellar worm through the foramen magnum. The animal did not show changes in complete blood count, biochemical series, and cerebrospinal fluid and was negative for Pestivirus. There was a worsening of the clinical conditions and the animal died. This malformation of unknown etiology must be studied as a differential diagnosis of the nervous system disorders.(AU)

Este estudo caracterizou os achados clínicos, radiológicos, ultrassonográficos e necroscópicos de um caso de malformação de Arnold-Chiari tipo II em uma bezerra Gir no Brasil. O animal foi hospilatizado aos 60 dias de idade, apresentando decúbito esternal permanente, apêndice cutâneo na altura da quarta vértebra lombar e cifoescoliose da coluna vertebral torácica caudal e lombossacra. Ao exame radiográfico da coluna e do crânio, foram observadas espinha bífida e suspeita de hipoplasia occipital. Ao exame de mielografia com injeção de contraste lombar e atlanto-occipital, foi possivel visualizar a meningocele na altura da quarta vértebra lombar e achados em nível rombencefálico de aumento da pressão regional com falha de preenchimento do contraste na fossa posterior, na presença de nítida demarcação das circunvoluções do córtex cerebral e do espaço subaracnoide da medula espinhal cervical. Ao exame ultrassonográfico do cerebelo, foi observada insinuação do verme cerebelar através do forame magno. O animal não apresentou alterações em hemograma completo, série bioquímica e fluido cérebro-espinhal e foi negativo para Pestivirus. Houve uma piora do quadro clínico e o animal morreu. Essa malformação de etiologia desconhecida deve ser estudada como um diagnóstico diferencial.(AU)

Hemifacial Spasm Associated with Chiari Type I Malformation: Surgical Considerations and Case Report

Hemifacial spasm (HS) is a movement disorder characterized by paroxysmal and irregular contractions of the muscles innervated by the facial nerve. Chiari malformation type I (CM I) is a congenital disease characterized by caudal migration of the cerebellar tonsils, and surgical decompression of foramen magnum structures has been used for treatment. The association of HS with CM I is rare, and its pathophysiology and therapeutics are speculative. There are only a few cases reported in the literature concerning this association. The decompression of the posterior fossa for the treatment of CM I has been reported to relieve the symptoms of HS, suggesting a relation between these diseases. However, the possible complications of posterior fossa surgery cannot be underrated. We report the case of a 66-year-old patient, in ambulatory follow-up due to right HS, no longer responding to botulinum toxin treatment. Magnetic resonance imaging (MRI) of the skull revealed compression of the facial nerve and CM I. The patient underwent surgery for HS by neurovascular microdecompression of the facial nerve via right lateral suboccipital craniectomy, but presented significant clinical worsening in the postoperative period even though the cerebellum edema related to surgical manipulation was mild. Due to the clinical worsening, the patient underwent a median suboccipital craniectomy with decompression of the foramenmagnum structures. After this second surgery, the patient had progressive improvement and was discharged from the hospital for ambulatory care.

Management of Chiari I Deformity in Children and Adolescents: A Report from the Consensus Taskforce of the Brazilian Society of Pediatric Neurosurgery

Much controversy remains on the current management of Chiari I deformity (CID) in children, with many clinical, surgical and ethic-legal implications. The Brazilian Society of Pediatric Neurosurgery (SBNPed, in the Portuguese acronym) has put together a panel of experts to analyze updated published data on the medical literature about this matter and come up with several recommendations for pediatric neurosurgeons and allied health professionals when dealing with CID. Their conclusions are reported herein, along with the respective scientific background.

Series de casos de malformación de Arnold Chiari: Hospital Nacional Mario Catarino Rivas, 2008-2018 / Arnold Chiaris malformation case series: Hospital Nacional Mario Catarino Rivas, 2008-2018

La malformación de Arnold Chia-ri (MAC) es una enfermedad por primera vez descrita en 1883 y clasificada en 1891 en cuatro grupos.Se ha reportado una prevalencia de malformación de Chiari tipo 1 de 0.24-0.9% de los adultos que se realizan un resonancia magnética cerebral por cualquier motivo. Los sín-tomas se relacionan a hidrocefalia obstructiva, movimientos oculares anormales y mielomeningocele espinal. Su causa es multifactorial y las manifestaciones clínicas muy variadas. Se relaciona con síndromes de craneosinostosis, síndromes osteopáticos, desórdenes del tejido conectivo, anomalías vertebrales, anomalías cráneofaciales, síndrome de Williams, síndrome de Noonan y neurofibromatosis. El diagnóstico se realiza por resonancia magnética.Casos clínicos: Se presenta una serie de 3 casos de pacientes que acudieron al Hospital Mario Catarino Rivas (HMCR) en el periodo de 2008-2018. El motivo de consulta fue diferente en cada paciente, siendo: vómito, pérdida de fuerza muscular, disfagia, dolor en miembro superior derecho, paresia y parestesia de miembro inferior y ataxia, aumento del perímetro cefálico. El tipo de malformación fue tipo1 en dos casos, tipo2 en un caso.Solo dos de los pacientes se operaron, teniendo evolución posquirúrgica satisfactoria. Conclusión: La MAC es una patología poco estudiada en el país. No existe una forma de presentación global, por lo que siempre hay que tenerla en cuenta cuando se presente un paciente con alguna de esta sintomatología...(AU)

Severe Cerebellar Degeneration and Chiari I Malformation - Speculative pathophysiology based on a systematic review

SUMMARY BACKGROUND Symptomatic Chiari Type I Malformation (CM) is treated with posterior fossa decompression with or without duroplasty. We have noticed some cases with concomitant severe cerebellar ataxia due to cerebellar atrophy. The aim of this study is to review the literature of CM associated with severe cerebellar atrophy and discuss its potential physiopathology. METHODS A systematic literature review in the Pubmed Database was performed using the following key-terms: "cerebellar atrophy Chiari", and "cerebellar degeneration Chiari". Articles reporting the presence of cerebellar degeneration/atrophy associated with CM were included. RESULTS We found only six studies directly discussing the association of cerebellar atrophy with CM, with a total of seven cases. We added one case of our own practice for additional discussion. Only speculative causes were described to justify cerebellar atrophy. The potential causes of cerebellar atrophy were diffuse cerebellar ischemia from chronic compression of small vessels (the most mentioned speculative cause), chronic raised intracranial pressure due to CSF block, chronic venous hypertension, and association with platybasia with ventral compression of the brainstem resulting in injury of the inferior olivary nuclei leading to mutual trophic effects in the cerebellum. Additionally, it is not impossible to rule out a degenerative cause for cerebellar atrophy without a causative reason. CONCLUSIONS Severe cerebellar atrophy is found in some patients with CM. Although chronic ischemia due to compression is the most presumed cause, other etiologies were proposed. The real reasons for cerebellar degeneration are not known. Further studies are necessary.

RESUMO OBJETIVO A Malformação de Chiari (MC) tipo I sintomática é tratada através da descompressão da fossa posterior com ou sem duroplastia. Observamos alguns casos com ataxia cerebelar grave concomitante devido à atrofia cerebelar. O objetivo deste estudo é revisar a literatura sobre MC associada à atrofia cerebelar grave e discutir sua possível fisiopatologia. METODOLOGIA Conduzimos uma revisão sistemática da literatura no banco de dados Pubmed utilizando as seguintes palavras-chave: "cerebellar atrophy Chiari", e "cerebellar degeneration Chiari". Artigos sobre a presença de degeneração/atrofia cerebelar associada à MC foram incluídos. RESULTADOS Encontramos apenas seis estudos que discutiam diretamente a associação entre atrofia cerebelar e MC, com um total de sete casos. Nós adicionamos um caso da nossa própria prática para ampliar a discussão. Apenas causas especulativas foram descritas para justificar a atrofia cerebelar, entre elas: isquemia cerebelar difusa devido à compressão crônica de pequenos vasos (a causa especulativa mais citada), pressão intracraniana elevada crônica devido ao bloqueio de LCR, hipertensão venosa crônica e associação com platibasia com compressão ventral do tronco cerebral, resultando em lesão do núcleo olivar inferior e levando a efeitos tróficos mútuos no cerebelo. Além disso, não é possível descartar uma causa degenerativa para atrofia cerebelar sem motivos claros. CONCLUSÃO A atrofia cerebelar grave é encontrada em alguns pacientes com MC. A isquemia crônica causada por compressão é a causa mais apontada como suspeita, porém outras etiologias foram propostas. As reais causas da degeneração cerebelar não são conhecidas. Mais estudos são necessários.

Basilar invagination associated with chiari malformation type I: A literature review

Basilar invagination (BI) and Chiari malformation type I (CM-I) are very important anomalies that introduce instability and compression in the occipitocervical transition region and have complex clinical characteristics. These anomalies vary according to the affected structures. The present study revises current knowledge regarding the anatomy, anatomo-physiology, clinical manifestations, and radiological findings of these entities and the associated surgical treatment approaches. A bibliographic survey was performed through a search in the Medline, PubMed, SciELO, Science and LILACS databases. When associated, these craniovertebral malformations result in neurological deficits due to neural parenchyma compression; however, the presence of microtraumas due to repetitive lesions caused by the bulb and cervical marrow instability has been highlighted as a determinant dysfunction. Surgical treatment is controversial and has many technical variations. Surgery is also challenging due to the complex anatomical characteristics and biomechanics of this region. Nevertheless, advances have been achieved in our understanding of related mechanisms, and compression and atlantoaxial instability are considered key elements when selecting the surgical approach.

Presyrinx Associated with Post-Traumatic Hydrocephalus Successfully Treated by Ventriculoperitoneal Shunt / 대한신경손상학회지

Presyrinx consists of reversible spinal cord swelling without frank cavitation, as observed on T2 weighted magnetic resonance imaging (MRI). The condition may evolve into syringomyelia, but timely surgical interventions have achieved meaningful results. Here, we report the case of a 27-year-old woman who presented with headache, dizziness, and diplopia 2 months after suffering a mild head trauma. On MRI, hydrocephalus, downward herniation of the cerebellar tonsil, and a diffuse high signal change in the cervical spinal cord were detected. After insertion of a ventriculoperitoneal shunt, her neurological symptoms resolved, and she has had no signs of presyrinx recurrence for >4 years.

Arnold-Chiari Type 1 Malformation Mimicking Benign Paroxysmal Positional Vertigo / 대한평형의학회지

Arnold-Chiari malformation type 1 is a congenital disease characterized by herniation of the cerebellar tonsils through the foramen magnum. Most common clinical symptom is pain, including occipital headache and neck pain, upper limb pain exacerbated by physical activity or valsalva maneuvers. Various otoneurological manifestations also occur in patients with the disease, which has usually associated with dizziness, vomiting, dysphagia, poor hand coordination, unsteady gait, numbness. Patients with Arnold-Chiari malformation may develop vertigo after spending some time with their head inclined on their trunk. Positional and down-beating nystagmus are common forms of nystagmus in them. We experienced a 12-year-old female who presented complaining of vertigo related to changes in head position which was initially misdiagnosed as a benign paroxysmal positional vertigo.

Dyke-Davidoff-Masson syndrome and chiari type ii malformation / Síndrome de Dyke-Davidoff-Masson e malformação de chiari tipo II

Dyke-Davidoff-Masson Syndrome is a syndrome associated with refractory epilepsy. The Chiari II malformation is a complex congenital malformation of the brain. The authors report a case of a 15 years-old adolescent presenting Dyke-Davidoff-Masson syndrome and Chiari type II malformation association. This case demonstrates an unusual association in neuroimaging tests that indicates the need to evaluate associated diseases, such as myelomeningocele, corpus callosum dysgenesis and syringohydromyelia.

A Síndrome de Dyke-Davidoff-Masson é uma síndrome associada à epilepsia refratária. A malformação de Chiari II é uma malformação congênita complexa do cérebro. Os autores relatam um caso de uma adolescente de 15 anos apresentando a síndrome de Dyke-Davidoff-Masson associada à malformação de Chiari tipo II. Este caso demonstra uma associação incomum nos exames de neuroimagem que indica a necessidade de avaliar doenças associadas, como mielomeningocele, disgenesia do corpo caloso e a siringohidromielia.

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.

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).

Pyramidal and Proprioceptive Dysfunctions as the Main Neurologic Features in Symptomatic Type I Chiari Malformation Patients

Objective Broader access to magnetic resonance imaging (MRI) has increased the diagnosis of tonsillar ectopia, with most of these patients being asymptomatic. The early diagnosis and treatment of type I Chiari malformation (CM I) patients has impact on the prognosis. This study supplements information about the neurologic exam of symptomatic patients with CM I. Methods The sample was composed of 32 symptomatic patients with CM I diagnosed by a combination of tonsil herniation of more than 5 mm below the magnum foramen (observed in the sagittal T2 MRI) and at least one of the following alterations: intractable occipital headache, ataxia, upper or lower motor neuron impairment, sensitivity deficits (superficial and deep) or lower cranial nerves disorders. Results Occipital headache was the most frequent symptom (53.12%). During the physical exam, the most common dysfunctions were those from the pyramidal system (96.87%), followed by posterior cord syndrome (87.5%). Discussion In this study, patients became symptomatic around the fifth decade of life, which is compatible with previous descriptions. Patients withmore than 2 years of evolution have worse responses to treatment. Occipital headache, symptoms in the upper limbs, gait and proprioceptive disorders are common findings in patients with CM I. Conclusion Deep tendinous reflexes and proprioception disorders were the main neurologic features found in symptomatic CM I patients.

Alterações auditivas em recém-nascidos e lactentes com mielomeningocele / Hearing impairment in newborns and infants with myelomeningocele

RESUMO Objetivo Verificar a ocorrência de alterações auditivas em recém-nascidos e lactentes com mielomeningocele e/ou Síndrome de Arnold Chiari tipo II. Métodos Foram incluídos na amostra 160 neonatos atendidos em unidade de terapia intensiva neonatal, distribuídos em três grupos: Grupo Estudo 1, formado por 42 neonatos com Síndrome de Arnold Chiari tipo II; Grupo Estudo 2, constituído por 38 neonatos com mielomeningocele e Grupo Controle, com 80 recém-nascidos sem síndromes, malformações e/ou riscos para alteração auditiva. Todos os pacientes realizaram a triagem auditiva neonatal com emissões otoacústicas evocadas por estímulo transiente e foram submetidos à pesquisa do potencial evocado auditivo de tronco encefálico com estímulo clique. Quando houve falha nas emissões, a pesquisa dos limiares eletrofisiológicos foi realizada mediante potencial evocado auditivo de tronco encefálico com tone burst . A análise estatística foi feita por meio de testes paramétricos e os dados encontrados foram descritos nos resultados, considerando o nível de significância p<0,05. Resultados Houve maior ocorrência de perda coclear, alteração central e neuropatia auditiva nos grupos estudados. No que se refere aos valores médios das latências absolutas e dos intervalos interpicos, nos três grupos, por orelha, houve aumento das latências absolutas das ondas III e V e dos intervalos interpicos III-V e I-V , no grupo de lactentes com Síndrome de Arnold Chiari tipo II. Conclusão Recém-nascidos e lactentes com Síndrome de Arnold Chiari tipo II e mielomeningocele apresentaram maior ocorrência de perda coclear, espectro da neuropatia auditiva e alterações centrais.

ABSTRACT Purpose To characterize Auditory and oral language performance in children under 2 years of age with Hearing and Language Development Scale (EDAL-1) and to compare the results to those of hearing children up to two years of age. Methods This is an experimental study of descriptive nature. A total of 141 children were divided into two groups: control group with 92 normal hearing, and the experimental group with 49 deaf children followed audiologically during the first two years after implantation. Normal hearing children underwent auditory assessment and parents responded to EDAL-1. The children implanted, every three months, underwent otorhinolaryngological and speech-language evaluation, and the parents answered EDAL-1. Results In both groups we observed upward responses, with improvement as chronological and auditory age increases. Results obtained in the study group are statistically worse than in the control group, but with a tendency to grow. EDAL-1 was easy to apply and allowed to monitor the performance of the implanted children. Conclusion Oral and auditory performance in deaf children with cochlear implants was characterized by EDAL-1, and it was shown to be growing as the time of auditory stimulation increased, a fact also seen in hearing children. Despite this, the results of the hearing children were better than those of the deaf children implanted.

A Case of a Girl with Arnold-Chiari Type 1 Malformation with Precocious Puberty / 가정의학회지

A small percentage of individuals have the neurological anomaly of central precocious puberty (CPP). Common neurologic causes of CPP include a tumor or congenital lesions. Although Arnold-Chiari malformation can be caused by congenital or acquired causes, it is unusual in patients with CPP. We present the case of a girl aged 4.5 years who complained of breast budding. Her neurological examination and growth pattern were normal. She had no endocrinological abnormality, except for true precocious puberty. We performed brain magnetic resonance imaging, which showed an Arnold-Chiari type 1 malformation. Currently, this case represents the youngest girl who exhibited both Arnold-Chiari type 1 malformation and precocious puberty. Furthermore, it is likely that there is a meaningful association between the brain lesion and precocious puberty in this case.

Posterior fossa decompression with duraplasty in Chiari surgery: A technical note / Descompressão de fossa posterior com duroplastia no tratamento cirúrgico do Chiari: nota técnica

Summary Chiari malformation (CM) is the most common and prevalent symptomatic congenital craniocervical malformation. Radiological diagnosis is established when the cerebellar tonsils are located 5 mm or more below the level of the foramen magnum on magnetic resonance imaging (MRI). Surgical treatment is indicated whenever there is symptomatic tonsillar herniation or syringomyelia/hydrocephalus. The main surgical treatment for CM without craniocervical instability (such as atlantoaxial luxation) is posterior fossa decompression, with or without duraplasty. The authors describe in details and in a stepwise fashion the surgical approach of patients with CM as performed at the State University of Campinas, emphasizing technical nuances for minimizing the risks of the procedure and potentially improving patient outcome.

Resumo A malformação de Chiari (MC) é a malformação craniocervical congênita sintomática mais comum e prevalente. O diagnóstico radiológico é definido quando as tonsilas cerebelares estão localizadas pelo menos 5 mm abaixo do nível do forame magno na ressonância magnética (RM). Quando há hérnia tonsilar sintomática, siringomielia ou hidrocefalia, o tratamento cirúrgico é indicado. O principal tratamento cirúrgico para MC sem instabilidade craniocervical (como a luxação atlantoaxial) é a descompressão da fossa posterior com ou sem duroplastia. Os autores descrevem detalhadamente a abordagem cirúrgica de pacientes com MC realizada na Universidade Estadual de Campinas, enfatizando nuances técnicas para minimizar os riscos relacionados ao procedimento e melhorar os resultados pós-operatórios.

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).

Eosinophilic Meningitis after Bovine Graft Duraplasty for Arnold-Chiari Malformation Type 1 in a 33-year-old Man

Cases of pediatric eosinophilic meningitis following duraplasty with a bovine graft have been reported. These patients recovered following the surgical removal of the dural graft or steroid therapy. Decompression for Chiari malformation is a common procedure in both pediatric and adult neurosurgery. We describe the case of a 33-yr-old male patient with eosinophilic meningitis following Chiari decompression via bovine graft duraplasty. Cerebrospinal fluid (CSF) study showed 49 red blood cells/μL and 129 leukocytes/μL with 17% eosinophils. There was no evidence of infectious disease. To our knowledge, this is the first report of adult eosinophilic meningitis after bovine graft duraplasty in Korea.

Lesión como malformación de Chiari secundaria a quiste aracnoidal de la cisterna cuadrigémina / Chiari malformation-like lesion secondary to arachnoid cyst of the quadrigeminal cistern

Se presenta el caso de una mujer de 31 años de edad con cefalea y cervicalgia que se agravaba con la maniobra de Valsalva, presentando además mareos, encontrándose una malformación de Chiari secundario a un quiste aracnoideo cuadrigeminal. Después de efectuada la resonancia magnética diagnóstica, la paciente fue sometida a descompresión del agujero magno y extirpación del quiste cuadrigeminal, seguido por la resolución tanto de la malformación de Chiari y el quiste. Los síntomas desaparecieron después de la cirugía y han permanecido completamente resuelto hasta la actualidad. En pacientes adultos que presentan signos y síntomas de una malformación de Chiari debido a la compresión de la médula por las las amígdalas cerebelosas, la presencia de un quiste aracnoideo de cisterna cuadrigéminal es una rara patología asociada que puede ser tratada quirúrgicamente.

We report a rare case of a 31-year-old woman with headache and pain manifested by cervicalgia that worsened with the Valsalva maneuver and dizziness, who was found to have a Chiari malformation secondary to a posterior fossa arachnoid cyst. After magnetic resonance imagining (MRI), the patient was submitted to foramen magnum decompression and arachnoid cyst removal that were followed by resolution of both the Chiari malformation and the cyst. The symptoms disappeared after surgery and have remained completely resolved to the present day. In adult patients who present with signs and symptoms of Chiari malformation due to direct medulla compression by the tonsils, a quadrigeminal cistern arachnoid cyst is a rare associated pathology that can be treated surgically.