Introduction: A retained medullary cord (RMC) is a rare dysraphic malformation, recently described as a late arrest of secondary neurulation. RMC is also a severely tethering lesion. The critical role of intraoperative neurophysiology to safely manage a RMC has been only anecdotally reported.
Case report: We describe the case of a RMC in a 1.5-year-old child with Currarino syndrome. At surgery, an apparently normal-looking spinal cord, stretched and tethered by a lipoma to the level of S2-S3, was observed. The border between the functional conus and the non functional RMC was defined through neurophysiological mapping. The cord was sharply interrupted at this level and untethered. A specimen was sent for pathology, which confirmed the presence of glial and neural elements. The post-operative neurological exam was normal.
Conclusion: Neurosurgical procedure for RMC should only be rendered with intraoperative neurophysiological mapping, as the anatomical judgment would not suffice to allow a safe cutting of these "normal-looking" neural structures.