Retained medullary cord extending to a sacral subcutaneous meningocele.

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.