Abstract
Background
A retained medullary cord (RMC) is a rare closed spinal dysraphism with a robust elongated neural structure continuous from the conus and extending to the dural cul-de-sac. One case extending down to the base of a subcutaneous meningocele at the sacral level has been reported.
Clinical presentation
We report on three cases of closed spinal dysraphism, in which a spinal cord-like tethering structure extended out from the dural cul-de-sac and terminated at a skin-covered meningocele sac in the sacrococcygeal region, which was well delineated in curvilinear coronal reconstructed images of 3D-heavily T2-weighted images (3D-hT2WI). Intraoperative neurophysiology revealed the spinal cord-like tethering structure was nonfunctional, and histopathology showed that it consisted of central nervous system tissue, consistent with RMC. The tethering structure histologically contained a glioneuronal core with an ependymal-like lumen and smooth muscle, which may indicate developmental failure during secondary neurulation.
Conclusions
When the RMC extending to a meningocele is demonstrated with the detailed magnet resonance imaging including 3D-hT2WI, decision to cut the cord-like structure for untethering of the nervous tissue should be made under careful intraoperative neurophysiological monitoring.
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Acknowledgments
We thank Ann Turnley, PhD, from Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript. This work was partly supported by Research Foundation of Fukuoka Children’s Hospital.
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Murakami, N., Morioka, T., Shimogawa, T. et al. Retained medullary cord extending to a sacral subcutaneous meningocele. Childs Nerv Syst 34, 527–533 (2018). https://doi.org/10.1007/s00381-017-3644-2
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DOI: https://doi.org/10.1007/s00381-017-3644-2