Multiple coexistent dysraphic pathologies

Four distinct dysraphic anomalies were observed in a single child. While combinations of such anomalies are well recognised, quadruple dysraphic pathology, nevertheless, is extremely uncommon. To our knowledge, no previous cases have been reported in the literature. We present the management of a ch...

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Veröffentlicht in:Child's nervous system 2003-06, Vol.19 (5-6), p.376-379
Hauptverfasser: SOLANKI, Guirish A, EVANS, James, COPP, Andrew, THOMPSON, Dominic N. P
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Sprache:eng
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Zusammenfassung:Four distinct dysraphic anomalies were observed in a single child. While combinations of such anomalies are well recognised, quadruple dysraphic pathology, nevertheless, is extremely uncommon. To our knowledge, no previous cases have been reported in the literature. We present the management of a child with a concurrent segmental meningocele, a type-1 split cord malformation (SCM) associated with hemivertebrae, lipomyelomeningoceles in each hemicord of the SCM and a terminal myelocystocele, and we review the literature on potential mechanisms of dysmorphogenesis. Existing embryologic hypotheses for the dysraphic spectrum lack experimental evidence and studies in animal models. This case challenges the existing hypotheses and illustrates our incomplete understanding of human terminal spinal cord embryogenesis. Further studies on the morphogenetic basis for these anomalies are required.
ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-003-0728-y