Triple neural tube defect--cranium bifidum with rostral and caudal spina bifida--live evidence of multi-site closure of the neural tube in humans

The coexistence of three neural tube defects (NTDs) in a single child is an exceptional event. A review of the literature revealed nine published "double" NTD cases, but no cases of "triple" NTDs have been reported to date. The rare case of a two-year-old boy with three distinct...

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Bibliographic Details
Published in:Child's nervous system 2005-04, Vol.21 (4), p.331-335
Main Author: Tekk k, Ismail H.
Format: Article
Language:English
Subjects:
Child, Preschool
Encephalocele - pathology
Humans
Male
Meningomyelocele - pathology
Neural Tube Defects - complications
Neural Tube Defects - pathology
Review Literature as Topic
Skull - pathology
Spina Bifida Occulta - pathology
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Summary:The coexistence of three neural tube defects (NTDs) in a single child is an exceptional event. A review of the literature revealed nine published "double" NTD cases, but no cases of "triple" NTDs have been reported to date. The rare case of a two-year-old boy with three distinct NTDs is presented. The boy had a 17x15x15-cm(3) parieto-occipital encephalocele, a small cervical myelomeningocele, and a 11x11x8-cm(3) thoracolumbar myelomeningocele. Hydrocephalus and Chiari II malformation accompanied the NTDs. All three lesions were surgically treated with good cosmetic results and satisfactory neurologic outcome. Current neural tube closure theories and models are reviewed in an attempt to better understand this extremely unusual coexistence. The multi-site closure model is clearly more useful in our understanding of NTDs.
ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-004-1027-y