Lipoma não disráfico da medula espinal, muito raro, caracterizado por se localizar no interior da medula espinal. Não há qualquer defeito na dura-máter sobrejacente.
Introdução
O que você precisa saber de cara
Lipoma não disráfico da medula espinal, muito raro, caracterizado por se localizar no interior da medula espinal. Não há qualquer defeito na dura-máter sobrejacente.
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
Triagem neonatal (Teste do Pezinho)
A triagem neonatal permite diagnóstico precoce e início imediato do tratamento.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Lipoma espinhal intramedular não-disráfico
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Non-dysraphic intramedullary spinal cord lipoma of the child: Report of 3 cases.
Non-dysraphic intramedullary spinal cord lipomas (NDSCL) represent 1% of spinal cord tumors. They are less frequent than dysraphic spinal cord lipomas and clinical presentation is unspecific. There are no guidelines on surgical management. We report three observations of NDSCL in children, focusing on the clinical presentation, surgical management and postoperative outcome. The patients, one female and two males, aged from 5 months to 10 years presented with neurological deterioration, pain, spinal rigidity and in two cases, a subcutaneous mass. Spinal MRI found intradural lipomas without spina bifida, located in the cervico-thoracic area in all cases. The lipoma extended to the medulla oblongata in two cases and was in the lumbar region in the third. These lipomas were massive, requiring decompression surgery. Surgery confirmed the lipoma to be subpial. We performed debulking of the lipoma without attempting total resection, and with or without dural plasty and laminoplasty, followed by minerva cast in two cases, and avoidance of standing in the youngest. Satisfactory recovery occurred in all three cases. After a follow-up between 4 months and 9 years, the outcome was favorable in all cases, and no patient presented with secondary spinal deformation or lipoma progression. NDSCL is a rare entity, which often manifests with progressive pain and neurological deficits. In our experience, partial resection with or without dural plasty and laminoplasty has been associated with satisfactory postoperative outcomes and no recurrence of symptoms. We should be attentive to the risk of postoperative spinal deformity in these young patients.
Non-dysraphic extramedullary intradural spinal lipoma with neurocutaneous melanocytosis.
Intradural spinal lipomas are very rare and constitute less than 1% of all spinal tumors. Such tumors are usually associated with spinal dysraphism and occur mostly in the lumbosacral or cervical region. Intradural spinal lipomas tends to be intramedullary or subpial. Meningeal melanocytoma is further rarer cases that comprise less than 0.1% of cases. These usually occur in the fifth or fifth decade and chances of malignant transformation are high. Here, we report an extremely rare case (first to the best of our knowledge) of a 9 years female child who presented to us with rapid progressing paraparesis. She was operated and found to have an intradural purely extramedullary spinal lipoma without spinal dysraphism. Moreover, she had melanin pigment deposits all over her meninges which is further rare. On presentation, the patient was bedridden but after surgery, the patient improved and could walk without support. To the best of our knowledge, this is the first case of spinal cord lipoma in dorsal location along with melanin pigments in the meninges. We discuss the pathogenesis, presentation and management of intradural extramedullary spinal lipomas.
Non Dysraphic Intramedullary Spinal Cord Lipoma.
Among all intramedullary spinal cord lesions, intramedullary spinal cord lipomas account less than 1%. Non-dysraphic intramedullary lipoma is very rare. It is most commonly seen in cervicodorsal region followed by cervico bulbar, lumbar and sometimes multiple. Here we present a 17-year-old female who underwent MRI due to upper dorsal pain followed by progressive bilateral lower limb weakness which showed intramedullary lesion extending from T1-T9, involving eight vertebral segments with distal syrinx and features suggestive of lipoma. Patient underwent surgical decompression of lipoma. Patient had an uneventful post-operative period. Diagnosis confirmed by histopathology.
Unusual presentation of a giant thoracic spinal cord lipoma.
Non-dysraphic intradural spinal cord lipomas are rare, and true intramedullary cervical-thoracic lipomas are extremely rare. Spinal lipomas usually present with chronic, progressive myelopathic features. Unlike dysraphic lipomas, which are usually located in the lumbo-sacral region, non-dysraphic lipomas are usually located in the cervical or thoracic spine. We present an unusual case of a 21-year-old female who presented with four months of severe back pain, progressive spasticity, and weakness in the lower limbs. Magnetic resonance imaging (MRI) revealed a T1- and T2-hyperintense lesion between D4 and D6. This fatty intramedullary lesion had undergone evolution and a possible hemorrhagic infarct and cord compression. The patient underwent an urgent dorsal laminoplasty and total resection of this lesion, which histopathology indicated was a fibrous lipoma. Total resection is possible in such cases if a micro-surgical technique that includes neurophysiological monitoring is used.
Non-dysraphic intramedullary lipoma of the craniocervical region in an adult: Case report.
Intradural spinal lipomas with intracranial extension are very rare and are typically diagnosed in childhood. Radical surgical excision usually causes a high rate of morbidity because of the firm adherence between the lipoma and neural tissues. In this report, we present a case of craniocervical intradural intramedullary lipoma in an adult patient. The patient underwent surgery with excision of the mass, leaving a sheet of lipoma on the tumor bed.
Publicações recentes
Non-dysraphic intramedullary spinal cord lipoma of the child: Report of 3 cases.
Non Dysraphic Intramedullary Spinal Cord Lipoma.
Unusual presentation of a giant thoracic spinal cord lipoma.
Non-dysraphic extramedullary intradural spinal lipoma with neurocutaneous melanocytosis.
[Intramedullary lipoma: about a case].
📚 EuropePMCmostrando 6
Non-dysraphic intramedullary spinal cord lipoma of the child: Report of 3 cases.
Neuro-ChirurgieNon Dysraphic Intramedullary Spinal Cord Lipoma.
Neurology IndiaUnusual presentation of a giant thoracic spinal cord lipoma.
Spinal cord series and casesNon-dysraphic extramedullary intradural spinal lipoma with neurocutaneous melanocytosis.
British journal of neurosurgery[Intramedullary lipoma: about a case].
The Pan African medical journalNon-dysraphic intramedullary lipoma of the craniocervical region in an adult: Case report.
NeurocirugiaAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Lipoma espinhal intramedular não-disráfico.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Lipoma espinhal intramedular não-disráfico
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Non-dysraphic intramedullary spinal cord lipoma of the child: Report of 3 cases.
- Non-dysraphic extramedullary intradural spinal lipoma with neurocutaneous melanocytosis.
- Non Dysraphic Intramedullary Spinal Cord Lipoma.
- Unusual presentation of a giant thoracic spinal cord lipoma.
- Non-dysraphic intramedullary lipoma of the craniocervical region in an adult: Case report.
- [Intramedullary lipoma: about a case].
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:645359(Orphanet)
- MONDO:0958075(MONDO)
- Busca completa no PubMed(PubMed)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar