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

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Abstract

Objective

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.

Case report

The rare case of a two-year-old boy with three distinct NTDs is presented. The boy had a 17×15×15-cm3 parieto-occipital encephalocele, a small cervical myelomeningocele, and a 11×11×8-cm3 thoracolumbar myelomeningocele. Hydrocephalus and Chiari II malformation accompanied the NTDs. All three lesions were surgically treated with good cosmetic results and satisfactory neurologic outcome.

Conclusions

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.

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Correspondence to Ismail H. Tekkök.

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Tekkök, I.H. Triple neural tube defect—cranium bifidum with rostral and caudal spina bifida—live evidence of multi-site closure of the neural tube in humans. Childs Nerv Syst 21, 331–335 (2005). https://doi.org/10.1007/s00381-004-1027-y

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  • DOI: https://doi.org/10.1007/s00381-004-1027-y

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