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).

Neuroartropatia de Charcot secundária à siringomielia: relato de caso / Charcot neuroarthropathy by syringomyelia: case report

A neuroartropatia de Charcot é uma das complicações da siringomielia, podendo ocasionar deformidades osteoarticulares e incapacidade funcional, com comprometimento das atividades da vida diária. Relatamos um caso de paciente com neuroartropatia de Charcot do ombro associada a siringomielia e malformação de Arnold-Chiari tipo I, acompanhado no Instituto Nacional de Traumatologia e Orto-pedia do Rio de Janeiro, RJ, Brasil.

Charcot neuroartropathy is one of the complications of syringomyelia and can lead to joint deformity and disability, affecting patients in activities of daily living. We report a case of a patient with shoulder Charcot neuroarthropathy associated with syringomyelia and Arnold-Chiari malformation type I, from the National Institute of Traumatology and Orthopedics of Rio de Janeiro, RJ, Brazil.

Suboccipital craniectomy with or without duraplasty: what is the best choice in patients with Chiari type 1 malformation? / Craniectomia suboccipital com ou sem duroplastia: qual a melhor escolha em pacientes com malformação de Chiari tipo 1?

The best surgical treatment for Chiari malformation is unclear, especially in patients with syringomyelia. We reviewed the records of 16 patients who underwent suboccipital craniectomy at our institution between 2005 and 2008. Of the six patients who did not undergo duraplasty, four showed improvement postoperatively. Two patients without syringomyelia showed improvement postoperatively. Of the four patients with syringomyelia, three showed improvement, including two with a decrease in the cavity size. One patient showed improvement in symptoms but the syringomyelia was unchanged. The cavity size increased in the one patient who did not show improvement. Among the 10 patients who underwent duraplasty, improvements were noted in four of the five patients without syringomyelia and in all of the five with syringomyelia. There is a suggestion that patients with syringomyelia may have a higher likelihood of improvement after undergoing duraplasty.

A melhor opção de tratamento cirúrgico na malformação de Chiari é desconhecido, especialmente em paciente com siringomielia. Nós revisamos casos de 16 pacientes submetidos à craniectomia suboccipital em nossa instituição de 2005 à 2008. Dos 6 pacientes que não foram submetidos à duroplastia, quatro tiveram melhora pós operatória. Dois pacientes sem siringomielia tiveram melhora pós operatória. Dos quatro pacientes com siringomielia, três tiveram melhora, incluindo dois com diminuição do tamanho da cavidade. Um paciente teve melhora da sintomatologia mas a siringomielia não se modificou. Dez pacientes foram submetidos à duroplastia. Melhora foi observada em 4 de 5 pacientes sem siringomielia e 5 de 5 casos com siringomielia. Há uma sugestão de que pacientes com siringomielia possam ter uma maior taxa de melhora após serem submetidos à duroplastia.

Association of HTLV-I with Arnold Chiari syndrome and syringomyelia

HTLV-I is associated with a broad spectrum of manifestations, including tropical spastic paraparesis and adult T-cell leukemia/lymphoma. Arnold Chiari syndrome is a condition characterized by herniation of the cerebellar tonsils through the foramen magnum. This condition should be suspected in all patients with headache and impaired motor coordination. Syringomyelia is a developmental anomaly that leads to the formation of an intramedullary cavity. Its clinical presentation is classically characterized by syringomyelic dissociation of sensation, with suspended distribution in the proximal portion of the trunk and upper limbs and preservation in other regions. We report here a case of association of the three diseases, which is rare in clinical practice, illustrating the difficulty in the diagnosis and therapeutic management of these conditions.

Malformación de Arnold-Chiari tipo I, siringomielia, siringobulbia y atrapamiento del ventrículo IV / Type 1 Arnold Chiari malformation, syringomielia, syringobulbia and IV ventricle entrapment

La malformación de Arnold Chiari (MAC) fue descrita inicialmente por Hans Chiari en 1890. Existen cuatro tipos, de los cuales el tipo I es el más frecuente en adultos y se encuentra por la elongación de las amígdalas y las divisiones mediales del lóbulo inferior del cerebelo hacia las proyecciones cónicas que acompañan al bulbo raquídeo en el canal espinal. Su origen es congénito, aunque puede presentarse como una forma adquirida en el contexto de infecciones o tumores de fosa posterior. Clínicamente puede comportarse de manera asintomática o dar sintomatología de compresión de bulbo raquídeo. La siringomielia —cavitación de las áreas centrales de la médula espinal— se asocia a la MAC tipo I; en 75 a 85% de los casos se manifiesta con dolor, debilidad o parestesias de extremidades. El ventrículo IV atrapado se debe a un cortocircuito a los ventrículos laterales y se asocia a bloqueo de la circulación del líquido cefalorraquídeo. Se describe un caso de MAC tipo I asociado a siringomielia, siringobulbia y ventrículo IV atrapado.

Arnold-Chiari Malformation (ACM) was first described by Hans Chiari in 1890. Four types of this malformation are recognized, of those, type I is the most common among adults. It is caused by an elongation of the cerebellum into the conic projections that accompany the brain stem within the spinal channel. It is mostly congenital but [quot ]acquired[quot ] forms can be seen in the context of infections or posterior fossa tumors. Clinically, it can present as an asymptomatic finding, but it can produce brain stem compression. Syringomielia--cavitation of the brain stem central areas--is associated with MAC type I in 75 to 85% of cases. Clinical signs include pain, weakness, extremity parestesia. A shunt to the lateral ventricles can produce IV ventricle entrapment and is associated with cerebrospinal fluid blockage. We describe a case of MAC type I associated with the presence of syringomielia, syringobulbia and IV ventricle entrapment.

Retrospective study of patients with Chiari: malformation submitted to surgical treatment / Estudo retrospectivo de pacientes com malformação de Chiari tratados cirurgicamente

The Chiari malformation (CM) is characterized by variable herniation of one or both cerebellar tonsils, associated or not with displacement of the hindbrain structures into the vertebral canal. This is a retrospective study of 29 patients with CM submitted to surgical procedure between 1990 and 2003. There were 14 females and 15 males whose ages ranged from 16 to 65 years. There were seven patients with isolated CM, 12 associated with syringomyelia (SM), three associated with basilar impression (BI) and seven associated with SM and BI. The surgery was based on posterior fossa decompression. In seven patients a catheter was introduced from the subarachnoid space into the III ventricle and five were submitted to tonsillectomy. Twenty-one patients improved, one worsened, one remained unchanged, four missed follow up and two died. We conclude that the best results with CM surgery are obtained by an effective posterior fossa decompression. Those CM cases associated with other abnormalities, such as SM and BI, probably need complementary techniques which will be the theme for new prospective studies.

A malformação de Chiari (MC) é o deslocamento variável de uma ou ambas as tonsilas cerebelares para o canal vertebral. Este trabalho é um estudo retrospectivo de pacientes com MC tratados com cirurgia de 1990 até 2003. Foram analisados 29 prontuários, sendo 15 pacientes do sexo masculino e 14 do feminino, com idade variando entre 16 a 65 anos. Destes, sete só apresentavam MC, 12 tinham MC associada com siringomielia (SM), três associada com impressão basilar (IB) e sete associada com SM e IB. Foi realizada descompressão da fossa posterior e plástica da dura-máter em todos. Em sete pacientes, foi acrescida a colocação de catéter do espaço subaracnóideo para o interior do IV ventrículo e em cinco a tonsilectomia. Ocorreu melhora dos sintomas em 21 pacientes, um permaneceu inalterado, houve piora em um caso, quatro não tiveram seguimento e dois faleceram. Conclui-se que a boa evolução da MC está relacionada com a descompressão efetiva da fossa posterior. Os casos de MC associadas a outras malformações, como IB e SM, talvez necessitem variações técnicas, as quais serão temas de novos estudos prospectivos.

Amigdalopexia cerebelosa en el manejo de la malformación de chiari de tipo I / Cerebellar amygdalopexy in the management of Chiari I malformation

Antecedentes. El tratamiento de la malformación de Chiari de tipo I asociada a siringomielia es controvertido. En este trabajo se presenta un análisis clínico, quirúrgico y radiológico de los pacientes con esta afección que fueron manejados durante un periodo de doce años. Material y métodos. Se incluyeron 48 pacientes, donde se encontró un discreto predominio en el sexo femenino. El cuadro clínico estuvo dominado por cefalea, dolor cervical, signos y síntomas cerebelosos, afección de nervios craneales bajos y lesión de la vía piramidal. Resultados. Tanto el grado de descenso amigdalino como el tamaño de la siringomielia fueron muy variables, sin encontrar correlación entre ambos. La cirugía consistió en una craniectomía occipital, laminectomía de C1, ascenso de amígdalas cerebelosas mediante coagulación bipolar y plastía de duramadre. Los mejores resultados clínicos se obtuvieron en el dolor y los síntomas cerebelosos, mientras que los peores fueron en la afección de los nervios craneales bajos y de la vía piramidal, sin embargo, en la gran mayoría de los pacientes se logró detener la progresión de los síntomas. No se presentaron complicaciones serias en el presente estudio. Conclusión. El procedimiento propuesto ofrece una alternativa segura, efectiva y comparable con otros métodos más riesgosos para el manejo de esta malformación.

BACKGROUND: The treatment of Chiari I malformation associated with syringomyelia is controversial. OBJECTIVE: We describe a series of patients with this disease treated during a twelve-year period. We also present clinical, surgical and radiological findings. MATERIAL AND METHODS: Forty eight patients were included; a non significant female predominance was found. Clinical course was characterized by headache, cerebellar signs and symptoms, neck pain and involvement of lower cranial nerves and pyramidal tract. The degree of tonsillar descent and syringomyelia size varied and a correlation between them was not found. Surgery consisted in an occipital craniectomy, C1 laminectomy and tonsillar elevation through bipolar coagulation and duraplasty. RESULTS: The best results were observed in pain and cerebellar symptoms, while a deficit of lower cranial nerves and pyramidal tract were observed. However, in most patients we were able to slow symptom progression. No adverse effects were documented. CONCLUSIONS: The surgicalprocedureproposed herein is an effective and safe treatment alternative for this malformation, and its results are comparable to other riskier procedures.

Cystic cervical intramedullary schwannoma with syringomyelia.

We report a case of cervical intramedullary cystic schwannoma associated with segmental syrinx in a young adult without evidence of neurofibromatosis. The relevant literature is reviewed.

Factors influencing the outcome in symptomatic Chiari I malformation.

BACKGROUND: To study the clinico-radiological determinants of outcome in patients with Chiari I malformation (CIM). MATERIALS AND METHODS: The disability assessment of 48 patients with C I M who underwent posterior decompression was done by modified Klekamp and Samii scoring system. The outcome was regarded as good when the patient was ambulant without any aid with an improvement in the disability score; and, poor when (a) there was postoperative deterioration or lack of improvement; (b) the patient was non-ambulant without aid, irrespective of the improvement in the clinical score; or, (c) there was a perioperative mortality. Patients with hydrocephalus; those who underwent syringo-subarachnoid or syringo-peritoneal shunt as the primary procedure; and, patients with atlanto-axial dislocation were excluded from the study. STATISTICAL METHODS USED: Categorical data were expressed in proportions and analyzed with Chi square test. Analysis of factors predicting clinical outcome at 6 months was done utilizing logistic regression analysis. RESULTS: The outcome assessed at six months showed that 30 patients (62.5%) had good outcome while 18 patients (37.5%; including two perioperative mortalities) had a poor outcome. CONCLUSIONS: Significant predictors of outcome in patients with CIM include the duration of symptoms (P value=0.006), respiratory distress (P value=0.001), and basilar invagination (P value=0.048). The effect of syringomyelia in predicting the clinical outcome could not be determined due to the differences in the number of patients in the groups with or without syringomyelia.

Results of the treatment of syringomyelia associated with Chiari malformation: analysis of 60 cases

Analisamos os resultados do tratamento cirúrgico de 60 pacientes com a associação de siringomielia (SM) e malformação de Chiari (MC) operados no período de 1982 a 2000. Em cada caso foram analisados 15 sinais e 16 sintomas, incluídos em um protocolo que separou os sinais e sintomas decorrentes da SM dos sinais e sintomas decorrentes da MC. Paralelamente ao protocolo, foi elaborado um sistema de pontuação que facilitou a avaliação dos resultados do tratamento. Todos os casos foram submetidos à descompressão crânio vertebral com laminectomia de C1 e eventualmente de C2, e tonsilectomia cerebelar com plástica de dura-máter. Para avaliação dos resultados, foram aplicados testes estatísticos de diferença de proporções e análise de variância com confiabilidade de 95% (p=0,05). Concluímos que os sinais e sintomas da MC apresentaram melhora estatística muito significativa (p=0). Os sinais e sintomas decorrentes da SM também apresentaram melhora significativa, sendo a exceção o sinal "hiporreflexia de membros superiores" que não melhorou. Não encontrada diferença estatística da melhora dos sintomas da SM quando comparados aos da MC. Os sinais na SM melhoraram estatisticamente mais que os sinais da MC. A metade dos pacientes apresentou percentual de melhora de seus sinais e sintomas na faixa entre 40 e 60%.

A patient with optic pathway glioma, scoliosis, Chiari type I malformation and syringomyelia: is it Neurofibromatosis type 1?

A 22 years old girl had features of optic pathway glioma, scoliosis, Chiari type 1 malformation and cervical syringomyelia. She had no cutaneous lesions. We considered this combination to be more than coincidental and argue in favour of considering the case as a variant form of Neurofibromatosis type 1. The relevent literature in favour of our contention has been reviewed.

Localized hypertrophy of hand in syringomyelia.

Segmental amyotrophy is a common feature resulting from extension of the syrinx into the anterior horns. Segmental hypertrophy in the form of chiromegaly/podomegaly or limb and hemi-hypertrophy are rare but well recognized clinical feature of syringomyelia. We report a case of hand hypertrophy secondary to syringomyelia. Hypertrophy of the hands is a rare but interesting feature of syringomyelia and in all cases of enlargements of hands syringomyelia should be considered in the differential diagnosis.

Retrocerebellar arachnoid cyst with syringomyelia: a case report.

Association of syringomyelia with retrocerebellar arachnoid cysts is rare. A case of 14 year old female is being reported, who presented with hydrocephalus caused by a large midline retrocerebellar infravermal arachnoid cyst leading to obstruction of the outlet foramina of the fourth ventricle. There was associated syringomyelia. The pathogenesis of syringomyelia is discussed. The need to evaluate cervical spinal cord by taking T1 weighted sagittal sections in all the patients of large posterior fossa mass lesions causing obstruction to the outlet foramina of the fourth ventricle has been stressed, in order to detect associated syringomyelia.

Lipomatosis múltiples simétricas / Multiple symmetric lipomatosis

Antecedentes: Los lipomas cervicales solitarios no requieren cirugía, salvo que por su volumen produzcan deformidades o dificultades mecánicas. Con las lipomatosis múltiples simétricas existe la controversia entre quienes prefieren la cirugía en aras de una mejor calidad de vida y quienes tratan de evitarla, basados en la incidencia de recurrencias. Objetivo: Analizar los aspectos sobresalientes de la enfermedad y los resultados alcanzados con la conducta adoptada. Lugar de aplicación: Hospital de Agudos y Hospital Oncológico, ambos de atención terciaria. Diseño: Retrospectivo no randomizado. Población: Se analizan 11 enfermos masculinos con una edad media de 51,6 años (44-63). La distribución era generalizada y comprensiva en el 54,5 por ciento. El hábito alcohólico se registró en el 100 por ciento. Métodos: En todos se practicó cirugía radical de los lipomas dominantes o que producían sintomatología comprensiva. En el 72,7 por ciento la resección cervical fue en un tiempo. Resultados: Se produjeron 3 complicaciones menores que se resolvieron sin nuevas cirugía. Sólo 1 (9 por ciento) recidivó. Al cierre de la observación, 8 enfermos se hallan vivos y sin recurrencias. Conclusiones: La cirugía radical es segura y exenta de riesgos. Las recurrencias se relacionan con el grado de meticulosidad de la cirugía

Syringohydromyelia or HTLV-I associeted myelopathy/tropical spastic paraparesis: a diagnostic challenge (case report)

Human T-cell lumphotropic virus type I (HTLV-I) associated myelopathy / tropical spastic paraparesis (HAM/TSP) is the most common chronic myelopathy in Brazil. We present the case of a 53 year old man that fulfiled the diagnostic criteria for HAM/TSP but had at the magnetic resonance imaging (MRI) of the spinal cord evidences of syringohydromyelia at the C6-C7 and D2-D7 levels along with Chiari type I malformation. The clinical picture was more typical of HAM/TSP than of syringohydromyelia, which was probably asymptomatic. The present case clearly demonstrates that sorology and neuroimaging should be always use together. We conclude that, specially in places where HTLV-I is endemic, every patient with a spatic paraparesis, even with a radiological picture suggestive of a structural spinal cord lesion, should have a screening test for HTLV-I. The clinical picture must dictate the final direction of the diagnosis.