ABSTRACT
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.
ABSTRACT
OBJECTIVE: To analyze the association between basilar invagination (BI) and stenosis in the hypoglossal canal (HC). METHODS: A case-control study with magnetic resonance images (MRIs) of the head from a local database was performed. The study used MRIs of 31 patients with BI (type B) and 36 controls, both groups over 18 years of age and without sex distinction. The internal (ID) and external (ED) diameters of the HC were measured on the coronal plane using the Osirix in its free version 3.9.2 (Mac-Apple platform). We used the Kolmogorov-Smirnov test (with Lilliefors adjustment) to evaluate the normality of the variables, the Levine test to verify the homogeneity of the variances, and Student's t test to verify differences between groups. All analyses were within the 95% confidence interval. RESULTS: Control group presented right and left ED values of 4.7 ± 0.8 mm and 4.6 ± 0.9 mm, respectively, while the right and left ID showed 4.4 ± 0.9 mm and 4.3 ± 0.8 mm, respectively. The group with BI showed right and left ED values of 3.3 ± 0.9 mm and 3.1 ± 0.9 mm, and the right and left ID had values of 2.8 ± 0.7 mm and 2.7 ± 0.7 mm, respectively. Both ED and ID were smaller in the group with BI (P < 0.001). CONCLUSIONS: Patients with BI of type B presented the narrowing of HC when compared with control participants.
Subject(s)
Foramen Magnum/abnormalities , Platybasia/pathology , Adult , Aged , Atlanto-Occipital Joint/abnormalities , Case-Control Studies , Constriction, Pathologic , Female , Humans , Hypoglossal Nerve , Male , Middle AgedABSTRACT
BACKGROUND: Craniometric studies document different subtypes of craniocervical junction malformations (CCJM). Here, we identified the different types and global signs and symptoms (SS) that correlated with these malformations while further evaluating the impact of syringomyelia. METHODS: Prospective data concerning SS and types of CCJM were evaluated in 89 patients between September 2002 and April 2014 using Bindal's scale. RESULTS: The mean Bindal's scores of each type of CCJM were Chiari malformation (CM) = 74.6, basilar invagination Type 1 (BI1) = 78.5, and BI Type 2 (BI2) = 78. Swallowing impairment and nystagmus were more frequently present in the BI patients. Symptomatic burdens were higher in patients with syringomyelia and included weakness, extremity numbness, neck pain, dissociated sensory loss, and atrophy. CONCLUSION: There were no statistically significant differences in SS between the different CCJM types. BI patients had more swallowing and nystagmus complaints versus CM patients, but there were no significant differences in clinical SS between BI1 and BI2 patients. Notably, those with attendant syringomyelia had a higher SS burden.
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BACKGROUND: The flexion of the skull base (basal angle [BA]) is the inclination between the anterior fossae and the basilar ramp of the occipital and sphenoid bones. An increased BA, termed platybasia, is usually associated with basilar invagination. BA reference values in the magnetic resonance imaging (MRI) era and the diagnosis of platybasia are of clinical importance. The transnasal approach has been the surgical technique of choice to remove the odontoid process in cases of ventral brainstem compression in patients with platybasia. The knowledge of normal BA values has been influenced by technological image acquisitions. The aim of this study was to determine the normal BA values in normal subjects in the MRI era. METHODS: For the determination of normal BA values, the literature reporting these values in normal individuals was reviewed and a meta-analysis of pertinent studies was performed. RESULTS: A total of 8 articles were included in this study, evaluating a total of 667 individuals. A summary measure of the results was obtained. The data obtained in this analysis provided a final basal angle value of 116.5 degrees (95% confidence interval, 104.39-128.7). The normal basal angle ranges between 104 and 129 degrees. CONCLUSION: Platybasia can be defined as a value >129 from the basal angle.
Subject(s)
Magnetic Resonance Imaging/methods , Neurosurgical Procedures/methods , Platybasia/diagnostic imaging , Platybasia/surgery , Skull Base Neoplasms/diagnostic imaging , Skull Base Neoplasms/surgery , Skull Base/diagnostic imaging , Skull Base/surgery , Humans , Reference ValuesABSTRACT
Down syndrome (DS) occurs when an individual has a full or partial extra copy of chromosome 21 and is the most common of all malformation syndromes. Associating with numerous pathologies like anterior atlantoaxial subluxation (AAAS) which is an increase in the space between the anterior arch of the first cervical vertebra (C1) and the odontoid process of the second vertebra (C2), most of the time its asymptomatic, only 1-2% to all the 30% who may have the AAAS and DS develop clinical symptoms. In this occasion, we present the case of a patient with SD and Juvenile chronic arthritis (JCA) who has atlantoaxial subluxation of approximately 11mm, basilar impression and platybasia with severe medullary compression in 2 points, requiring surgical management by the Neurological Surgery Service in Bucaramanga-Colombia.
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We report a rare case of trigeminal neuralgia. A 23-year-old woman with a history of 1 year of typical trigeminal neuralgia manifested the characteristics of basilar impression. Magnetic resonance imaging (MRI) demonstrated basilar impression, deformity of the posterior fossa with asymmetry of petrous bone, and compression of medulla oblongata in the topography of the odontoid apophysis. The operation was performed through a suboccipital craniectomy. The neuralgia disappeared after surgery and remains completely resolved until today. This is the second reported case of trigeminal neuralgia in a patient with basilar impression in Brazil.
ABSTRACT
The prime objective in the surgical treatment of basilar impression (BI), Chiari malformation (CM), and/or syringomyelia (SM) is based on restoration of the normal cerebrospinal fluid (CSF) dynamics at the craniovertebral junction and creation of a large artificial cisterna magna, avoiding the caudal migration of the hindbrain. It is observed that a large craniectomy might facilitate an upward migration of the posterior fossa structures. There are many surgical techniques to decompress the posterior fossa; however, a gold standard approach remains unclear. The authors present the results of 192 cases of BI, CM, and SM treated between 1975 and 2008 and whose surgical treatment was characterized by a large craniectomy without tonsillectomy with the patient in the sitting position, large opening of the fourth ventricle, and duraplasty.
O principal objetivo no tratamento cirúrgico da impressão basilar, malformação de Chiari e/ou siringomielia fundamenta-se na restauração da dinâmica do líquido cefalorraqueano ao nível da transição craniovertebral e criação de cisterna magna ampla. Isto é fator importante para evitar a migração caudal das estruturas da fossa posterior. A craniectomia ampla facilita a migração cranial dessas estruturas. Existem várias técnicas cirúrgicas para descomprimir a fossa posterior, mas não há evidência sobre qual a melhor.. Os autores apresentam os resultados de 192 casos de impressão basilar, malformação de Chiari e siringomielia, operados entre 1975 e 2008, nos quais o tratamento cirúrgico se baseou em ampla craniectomia com o paciente em posição sentada, sem tonsilectomia, abertura ampla do quarto ventrículo e enxerto dural.
Subject(s)
Adolescent , Adult , Aged , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Young Adult , Arnold-Chiari Malformation/surgery , Craniotomy/methods , Fourth Ventricle/surgery , Platybasia/surgery , Syringomyelia/surgery , Dura Mater/surgery , Retrospective Studies , Treatment OutcomeABSTRACT
The prime objective in the surgical treatment of basilar impression (BI), Chiari malformation (CM) and/or syringomyelia (SM) is based on the restoration of the normal cerebrospinal fluid (CSF) dynamics at the craniovertebral junction through the creation of a large artificial cisterna magna. A small suboccipital craniectomy has been emphasized to avoid caudal migration of the hindbrain structures into the vertebral canal. Nevertheless, the results showed downward migration of the hindbrain related to that type of craniectomy. The authors present, otherwise, the results of 104 cases of BI, CM and/or SM, whose surgical treatment was characterized by a large craniectomy with the patient in the sitting position, tonsillectomy, large opening of the fourth ventricle and duraplasty with creation of a large artificial cisterna magna. A significant upward migration of the posterior fossa structures was detected by postoperative magnetic resonance imaging.
O principal objetivo no tratamento cirúrgico da impressão basilar, malformação de Chiari e/ou siringomielia fundamenta-se na restauração dinâmica do líquido cefalorraqueano ao nível da transição craniovertebral e criação de ampla cisterna magna. Uma craniectomia suboccipital de pequenas dimensões foi proposta para evitar a migração caudal de estruturas rombencefálicas no canal vertebral. Entretanto, os resultados evidenciaram migração caudal do rombencéfalo. Os autores apresentam, por outro lado, os resultados de 104 casos de malformação de Chiari e/ou siringomielia, nos quais o tratamento cirúrgico se baseou em ampla craniectomia com o paciente em posição sentada, tonsilectomia, abertura ampla do quarto ventrículo e enxerto dural com consequente criação de ampla cisterna magna. Uma significante migração cranial das estruturas da fossa posterior foi detectada pelo emprego pós-operatório da ressonância magnética.