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Diastematomyelia is a rare congenital disorder characterized by the separation of the spinal cord by an osseocartilaginous or fibrous septum. While diastematomyelia has been reported to be more common in the thoracic and lumbar regions, the true incidence of cervical diastematomyelia is currently unknown. In this study, we conducted the most comprehensive systematic review to date of all other case reports of diastematomyelia to better characterize the incidence of cervical diastematomyelia, and provide comprehensive statistics on the clinical characteristics of diastematomyelia generally. Ninety-one articles were included in our study, comprised of 252 males (27.9%) and 651 females (72.0%) (and one patient with unspecified gender). In 507 cases, the vertebral level of the diastematomyelia was described, and we recorded those levels as either cervical (n=8, 1.6%), thoracic (n=220, 43.4%), lumbar (n=277, 54.6%), or sacral (n=2, 0.4%). In 719 cases, the type of diastematomyelia was specified as either Type I (n=482, 67.0%) or Type II (n=237, 33.0%). Our study found that diastematomyelia has been reported in the cervical region in only 1.6% of cases, and we provide comprehensive data that this disorder occurs in females to males with an approximately 2.6:1 ratio, and Type I vs. Type II diastematomyelia in an estimated 2:1 ratio.
Diastematomyelia is a rare congenital disorder characterized by the separation of the spinal cord by an osseocartilaginous or fibrous septum. While diastematomyelia has been reported to be more common in the thoracic and lumbar regions, the true incidence of cervical diastematomyelia is currently unknown. In this study, we conducted the most comprehensive systematic review to date of all other case reports of diastematomyelia to better characterize the incidence of cervical diastematomyelia, and provide comprehensive statistics on the clinical characteristics of diastematomyelia generally. Ninety-one articles were included in our study, comprised of 252 males (27.9%) and 651 females (72.0%) (and one patient with unspecified gender). In 507 cases, the vertebral level of the diastematomyelia was described, and we recorded those levels as either cervical (n=8, 1.6%), thoracic (n=220, 43.4%), lumbar (n=277, 54.6%), or sacral (n=2, 0.4%). In 719 cases, the type of diastematomyelia was specified as either Type I (n=482, 67.0%) or Type II (n=237, 33.0%). Our study found that diastematomyelia has been reported in the cervical region in only 1.6% of cases, and we provide comprehensive data that this disorder occurs in females to males with an approximately 2.6:1 ratio, and Type I vs. Type II diastematomyelia in an estimated 2:1 ratio.
Diastematomielia é um disrafismo espinhal oculto (HSD), descrito pela primeira vez em 1837 e representa 3,8% de todos os HSD. Poucos casos de diastematomielia cervical foram relatados, uma vez que a localização principal da doença é a coluna torácica baixa, no cone medular. Neste artigo, fizemos uma revisão da literatura e descrevemos um caso raro de diastematomielia cervical associada a lipoma e hipotrofia de membros superiores. Relato do caso: Uma mulher de 48 anos apresentou-se em nosso ambulatório com queixa de atrofia da porção distal do membro superior direito, que evoluiu para o lado contralateral após seis meses. No intraoperatório, a membrana epidural foi aberta, sendo encontrada uma lesão amarelada, reforçando o diagnóstico sugestivo de lipoma. Após a remoção, a medula espinhal dividida tornou-se clara. Discussão: Segundo nossa pesquisa, este é o primeiro caso de lipoma associado a diastematomielia na região cervical. Desde 1985, apenas 23 pacientes adultos foram diagnosticados com exames de imagem e tratados cirurgicamente. É possível dividir os pacientes com malformação da medula espinhal (SCM) em dois grupos. Um grupo de pacientes diagnosticados nos primeiros anos de vida com sintomas remanescentes e outro grupo formado por aqueles diagnosticados na idade adulta. Nosso paciente encontra-se no segundo grupo.
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