Diastematomyelia in 172 Children: The Impact of Modern Neuroradiology

Harwood-Nash, Derek C.; McHugh, Kieran

doi:10.1159/000120535

Cited by 51 publications

(47 citation statements)

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“…Among these, the present case is the only one with a dorsally attached bony spur. Extensive search in the English literature has not revealed a similar case [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19]. Even the recently published series on SCM does not mention such an entity [14].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Dorsally Situated Bony Spur in a Lumbar Split Cord Malformation

Chandra

Kamal

Mahapatra³

1999

Pediatr Neurosurg

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A case of lumbar split cord malformation (SCM) with a bony spur situated dorsally is presented. This was associated with a hypertrophied posterior arch. The ventral dura was totally intact, and there was no fibrous septum connecting the bony arch to the dura. To our knowledge, such a case has not been reported earlier. In view of this unique finding, we propose a slight modification in Pang's unified theory of embryogenesis in the development of SCM.

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Section: Discussionmentioning

confidence: 99%

An Unusual Case of Dorsally Situated Bony Spur in a Lumbar Split Cord Malformation

Chandra

Kamal

Mahapatra³

1999

Pediatr Neurosurg

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“…Pang [1] reported on 2 cases of composite-type SCM among 39, and Erşahin et al [15] reported on 4 cases of this type among 74. The frequency of composite-type SCM was less than 1% in the series reported by Harwood-Nash and McHugh [16]. Gower et al [17] had only 1 patient with composite-type SCM with a bone spur at the midcleft and another at the caudal end of the cleft.…”

Section: Discussionmentioning

confidence: 99%

Long-Segment Type 1 Split Cord Malformation with Two-Level Split Cord Malformation and a Single Dural Sac at the Lower Split

Singh

Khandelwal²,

Singh³

et al. 2011

Pediatr Neurosurg

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Objective and Importance: Two-level bony spurs are rare and also a very long segment of bony spur is very rare. Bony spur with a single dural covering is not reported in the literature. Here, we report a case of prophylactic surgery performed on a patient with a bony spur with a single dural covering who showed a good postoperative outcome. This case highlights the importance of prophylactic surgery in such patients to avoid cord injuries. Clinical Presentation: Since birth a 1-year-old child had had a tuft of hair at the dorsal (D₆ and D₇) region. The patient was examined and found to have scoliosis with convexity towards the right. There was no neurological deficit. MRI and CT spine scans showed 2-level type 1 split cord malformation (SCM) and there was a low-lying conus at the L_3–4 level. Intervention: The patient was operated on at the age of 1 year and 2 separate bony septae were observed. The upper one extended from D₅ to D₉ and the lower one was at the D₁₁ level. The bony spurs were excised. The filum was sectioned at the L_4–5 level by a separate incision. The patient had a good postoperative recovery. Conclusion: With meticulous planning and careful surgery, prophylactic surgery can have a very good outcome in long-segment and two-level type 1 SCM.

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“…Esta alteração é caracterizada pela divisão da medula no plano sagital com separação ou não do saco dural e pode estar associada a várias outras anomalias como mielomeningocele, seio dérmico, seringomielia, lipomas e alterações ósseas, incluindo a escoliose. A diastematomielia é 2 a 3 vezes mais comum em pacientes do sexo feminino e é mais frequente na região lombar, mas pode estar localizada em todas as áreas da coluna incluindo a região cervical e a transição crânio-cervical 4,[8][9][10][11] . Em geral, é diagnosticada em crianças e os sintomas mais frequentes são aqueles relacionados às alterações ortopédicas, à bexiga neurogênica e ao déficit motor, que pode se apresentar com caráter progressivo, sugerindo síndrome da medula presa 11 .…”

Section: Discussionunclassified

“…Estas lesões cutâneas geralmente estão situadas na linha média mas não necessariamente correspondem ao nível de lesão da diastematomielia 12 . A radiografia simples de coluna é o exame indicado para a investigação inicial e evidencia imagem compatível com esporão ósseo em 20 a 60% dos casos 10,14 . Outras evidências incluem alterações das lâminas, como fusão vertical, e dos pedículos com separação deles ou diminuição da sua altura; hemivértebras, fusão do corpo vertebral, redução do tamanho das vértebras e duplicação ou ausência de processos espinhosos.…”

Section: Discussionunclassified

Tumor dermóide intramedular associado a diastematomielia: relato de caso

Martins

Moraes

Siqueira

2000

Arq. Neuro-Psiquiatr.

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RESUMO -A associação de tumor dermóide intramedular e diastematomielia é rara, descrita anteriormente em 8 casos. Descrevemos novo caso, de um adulto portador desta associação que apresentou paraparesia crural progressiva e síndrome sensitiva nos membros inferiores, além de cifoescoliose tóraco-lombar. A exploração cirúrgica confirmou a ocorrência de um tumor dermóide intramedular além da diastematomielia identificados na ressonância magnética. Discutemos aspectos fisiopatológicos e clínicos e as características radiológicas destas patologias destacando a importância do diagnóstico precoce.PALAVRAS-CHAVE: diastematomielia, disrafismo espinhal, tumor espinhal. Intraspinal dermoid tumor associated with diastematomyelia: case reportABSTRACT -Intraspinal dermoid tumor and diastematomyelia is a rare combination of pathologies and only eight cases were priorly published. We report a new case in an adult with clinical presentation of progressive paresis and sensitive deficit in the lower limbs, associated with a thoraco-lombar kyphoscoliosis. The diagnosis based on the CT scan and MR images was confirmed at surgery. Pathophysiologic and clinical aspects as well as the radiological characteristics of these pathologies are dicussed and the importance of the early diagnosis is emphasized.KEY WORDS: diastematomyelia, spinal dysraphism, spinal tumor.Os tumores dermóides constituem de 1 a 2% de todos os tumores intrarraquidianos em todas as idades, apresentando localização intramedular em cerca de metade dos casos 1 . A associação de tumor dermóide com diastematomielia é rara, descrita anteriormente em 8 casos [1][2][3][4] . Neste relato apresentamos o caso de um paciente de 32 anos que apresentava déficit motor progressivo nos membros inferiores associado a deformidade da coluna tóraco-lombar. A investigação por imagem evidenciou processo expansivo intradural associado a alterações relacionadas a disrafismo espinhal incluindo diastematomielia. CASOJCA, masculino, 32 anos, natural de Cunha -SP, apresentou-se com queixa de 2 anos de déficit motor progressivo nos membros inferiores, associado a dor em queimação acometendo território de raízes L4 e L5 e alteração progressiva da curvatura da coluna lombar, notada desde o nascimento. No exame físico a estatura do paciente era 1, 44 m e, ao exame da região dorsal, notava-se lesão cicatricial radiada paravertebral esquerda com cerca de 8 cm de diâmetro e lesão puntiforme na linha mediana através da qual o paciente referia drenagem de secreção, não caracterizada, de forma intermitente, ao longo dos anos. O exame mostrava ainda anestesia tátil-térmica-dolorosa na face anterior da perna esquerda associada a hiperestesia na face anterior da coxa ipsilateral.

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Diastematomyelia in 172 Children: The Impact of Modern Neuroradiology

Cited by 51 publications

References 1 publication

An Unusual Case of Dorsally Situated Bony Spur in a Lumbar Split Cord Malformation

An Unusual Case of Dorsally Situated Bony Spur in a Lumbar Split Cord Malformation

Long-Segment Type 1 Split Cord Malformation with Two-Level Split Cord Malformation and a Single Dural Sac at the Lower Split

Tumor dermóide intramedular associado a diastematomielia: relato de caso

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