Uma condição rara, presente desde o nascimento (congênita), em que a medula espinhal é dividida ao meio por um tipo de tecido resistente, que pode ser fibroso ou até mesmo ósseo. Ela pode aparecer sozinha ou junto com a espinha bífida.
Introdução
O que você precisa saber de cara
Uma condição rara, presente desde o nascimento (congênita), em que a medula espinhal é dividida ao meio por um tipo de tecido resistente, que pode ser fibroso ou até mesmo ósseo. Ela pode aparecer sozinha ou junto com a espinha bífida.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 3 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 5 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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 — Malformação do cordão dividido tipo I
Centros de Referência SUS
24 centros habilitados pelo SUS para Malformação do cordão dividido tipo I
Centros para Malformação do cordão dividido tipo I
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
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
A clinical clue toward recognition of split cord malformation type-I resembling meningocele in neonates.
This is a letter to share the clinical experience we had with neonates born with a specific feature of spina bifida. The lesion is like a meningocele containing skim amount of fluid, with a tiny dimple around the center of the cutaneous lesion which causes inward retraction of the dysplastic coverage. This feature is in favor of accompanied split cord malformation (SCM) type I, and the central nidus is in continuous with the dural sleeve around the bony septum of SCM. By recognition of this clinical clue, surgical repair can be planned to perform with appropriate provision, particularly foreseeing the amount of intraoperative bleeding and duration of anesthesia in the newborn patients.
Complex spinal dysraphism: myelomenigocele associated with dorsal bony spur, split cord malformation type I, syringomyelia, lipoma and tethered cord.
Variations in split cord malformation (SCM) are known. However, association of SCM type I with myelomeningocele along with same level dorsal bony spur has not been described previously. We report a 1-year old male child who presented with these findings with associated syringomyelia, lipoma and tethered cord.
Treatment of spinal deformity with diastematomyelia type I: one-stage, two-stage surgery and new technique (vertebral column resection through wide bony septum).
Case series, literature review, and technical note. To compare two different approaches to treat the spinal deformity with split cord malformation type I (SCM I). To present a new method of one-stage surgical treatment of congenital spinal deformity with wide bony septum (SCM I). Analysis of the literature on the different types of combined surgical treatment of spinal deformities with SCM I was performed. We have provided our own data on 27 patients treated for congenital spinal deformity and SCM I, one of which underwent Schwab IV type osteotomy at the apex of the deformity through the bony septum and pedicles. Inclusion criteria were presence of spinal deformity in combination with SCM 1, performed surgery to correct spinal deformity, and follow-up period of at least 2 years. The result of the literature review was controversial and requires additional research. The average age of patients was 8.8 ± 6.6 years old. One-stage treatment of SCM I and spinal deformity was performed in 10 patients (group I) and two-stage in 14 patients (group II). Three patients with severe myelodysplasia, SCM I, and congenital kyphoscoliosis underwent correction of spinal deformity without SCM I removing (group III). The group I had the longest surgery duration (mean 289 ± 75 min) and largest blood loss (mean 560 ± 386 ml), a high percentage of deformity correction (mean 69.6%), and the highest rate of complications (60%). The most optimal was the two-stage treatment with the mean surgery duration 191 ± 137 min, mean blood loss 339 ± 436 ml, mean correction rate 63%, and frequency of complications 21%. The average follow-up time was 6.0 ± 2.6 years. One stage surgery associated with a large surgical invasion and a large number of complications. It can be used in some cases, for example when the wide bony septum (SCM I) is localized at the apex of the congenital scoliosis or kyphosis. In all other cases, it is worth adhering to a two-stage treatment. Many new works demonstrate the relative safety and effectiveness of deformity correction without removing the SCM. In our opinion, indications for treatment of spinal deformity without SCM I removing can be the need to perform a shortening ostetomy outside the SCM zone. The remaining cases require a thorough assessment and a balanced decision.
Lipomeningocele associated with diplomyelia in a dog.
A 2-year-old male neutered mixed breed dog with a body weight of 30 kg was presented for evaluation of a soft subcutaneous mass on the dorsal midline at the level of the caudal thoracic spine. A further clinical sign was intermittent pain on palpation of the area of the subcutaneous mass. The owner also described a prolonged phase of urination with repeated interruption and re-initiation of voiding. The findings of the neurological examination were consistent with a lesion localization between the 3rd thoracic and 3rd lumbar spinal cord segments. Magnetic resonance imaging revealed a spina bifida with a lipomeningocele and diplomyelia (split cord malformation type I) at the level of thoracic vertebra 11 and 12 and secondary syringomyelia above the aforementioned defects in the caudal thoracic spinal cord. Surgical resection of the lipomeningocele via a hemilaminectomy was performed. After initial deterioration of the neurological status postsurgery with paraplegia and absent deep pain sensation the dog improved within 2 weeks to non-ambulatory paraparesis with voluntary urination. Six weeks postoperatively the dog was ambulatory, according to the owner. Two years after surgery the owner recorded that the dog showed a normal gait, a normal urination and no pain. Histopathological diagnosis of the biopsied material revealed a lipomeningocele which confirmed the radiological diagnosis. Ein 2-jähriger Mischlingsrüde mit einem Körpergewicht von 30 kg wurde aufgrund einer dorsalen subkutanen Masse im Bereich der kaudalen Brustwirbelsäule vorgestellt. Ein weiteres klinisches Symptom war eine intermittierende Schmerzhaftigkeit bei Palpation im Bereich dieser Masse. Der Besitzer beschrieb zudem eine verlängerte Phase des Urinabsatzes mit wiederholter Unterbrechung und Wiederaufnahme der Miktion. Die Befunde der neurologischen Untersuchung entsprachen der Lokalisation der Rückenmarksabschnitte zwischen dem 3. Brust- und 3. Lendenwirbel. Die magnetresonanztomographische Untersuchung zeigte eine Spina bifida mit einer Lipomeningozele und Diplomyelie (Split-Cord-Malformation Typ I) auf Höhe des 11. und 12. Brustwirbels und eine sekundäre Syringomyelie kranial des besagten Defekts im Bereich der kaudalen Brustwirbelsäule. Es wurde eine chirurgische Resektion der Lipomeningozele mittels Hemilaminektomie vorgenommen. Nach anfänglicher postoperativer Verschlechterung des neurologischen Zustands mit Paraplegie und fehlender Tiefenschmerzwahrnehmung kam es innerhalb von 2 Wochen zu einer Verbesserung. Der Hund wies eine nicht gehfähige Paraparese auf und konnte selbstständig Urin absetzen. Nach Aussage des Besitzers war der Hund 6 Wochen postoperativ gehfähig. Zwei Jahre nach der Operation berichtete der Besitzer, der Hund zeige einen normalen Gang, einen unauffälligen Urinabsatz und keine Anzeichen für Schmerzen. Die histopathologische Untersuchung bestätigte die radiologische Diagnose einer Lipomeningozele.
Lateral lipomyelomeningocele of the hemicord with split cord malformation type I revealed by 3D heavily T2-weighted MR imaging.
Lipomyelomeningocele (LMMC) is defined by a low-lying tethered spinal cord protruding posteriorly from the spinal canal and terminating in a lipomatous mass in the subcutaneous meningeal sac. The coexistence of LMMC with split cord malformation (SCM) is rare. We report on a patient with laterally protruded LMMC arising from the hemicord of SCM type I. Direct coronal and axial views (instead of sagittal views) of 3D heavily T2-weighted MR imaging (3D-hT2WI) clearly demonstrated the topographical relationship between both of the hemicords, the bony septum, and nerve roots in the right subcutaneous meningeal sac. Combined use of axial and coronal images of 3D-hT2W is useful for visualization and surgery of such a complicated anomaly.
Publicações recentes
A clinical clue toward recognition of split cord malformation type-I resembling meningocele in neonates.
Treatment of spinal deformity with diastematomyelia type I: one-stage, two-stage surgery and new technique (vertebral column resection through wide bony septum).
Complex spinal dysraphism: myelomenigocele associated with dorsal bony spur, split cord malformation type I, syringomyelia, lipoma and tethered cord.
Lipomeningocele associated with diplomyelia in a dog.
Lateral lipomyelomeningocele of the hemicord with split cord malformation type I revealed by 3D heavily T2-weighted MR imaging.
📚 EuropePMC189 artigos no totalmostrando 7
A clinical clue toward recognition of split cord malformation type-I resembling meningocele in neonates.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryTreatment of spinal deformity with diastematomyelia type I: one-stage, two-stage surgery and new technique (vertebral column resection through wide bony septum).
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryComplex spinal dysraphism: myelomenigocele associated with dorsal bony spur, split cord malformation type I, syringomyelia, lipoma and tethered cord.
British journal of neurosurgeryLipomeningocele associated with diplomyelia in a dog.
Tierarztliche Praxis. Ausgabe K, Kleintiere/HeimtiereLateral lipomyelomeningocele of the hemicord with split cord malformation type I revealed by 3D heavily T2-weighted MR imaging.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryDorsal ectopic breast in a case of spinal dysraphism: A rare entity.
Neurology IndiaSplit cord malformation type I distal to segmental myelomeningocele.
Saudi medical journalAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Malformação do cordão dividido tipo I
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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.
- A clinical clue toward recognition of split cord malformation type-I resembling meningocele in neonates.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery· 2023· PMID 37310473mais citado
- Complex spinal dysraphism: myelomenigocele associated with dorsal bony spur, split cord malformation type I, syringomyelia, lipoma and tethered cord.
- Treatment of spinal deformity with diastematomyelia type I: one-stage, two-stage surgery and new technique (vertebral column resection through wide bony septum).Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery· 2022· PMID 34626222mais citado
- Lipomeningocele associated with diplomyelia in a dog.
- Lateral lipomyelomeningocele of the hemicord with split cord malformation type I revealed by 3D heavily T2-weighted MR imaging.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery· 2017· PMID 28247108mais citado
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:1671(Orphanet)
- OMIM OMIM:222500(OMIM)
- MONDO:0009106(MONDO)
- GARD:1851(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q3026420(Wikidata)
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
