Saccular Limited Dorsal Myeloschisis with Spinal Cord Deviation out of the Spinal Canal to the Sac

Saccular limited dorsal myeloschisis (LDM) is characterized by a fibroneural stalk linking the saccular skin lesion to the underlying spinal cord. Since untethering surgery during the early postnatal period is often indicated to prevent sac rupture, saccular LDM should be distinguished from myelomeningocele (MMC) during the perinatal period. We treated two patients with the spinal cord deviation from the spinal canal to the sac, which mimicked a prolapse of the neural placode into the MMC sac. In patient 1, pre- and postnatal magnetic resonance imaging (MRI) revealed that the spinal cord was strongly tethered to the thick stalk. During surgery, the dorsally bent cord and stalk were united, and the border between these two was determined with intraoperative neurophysiological mapping (IONM). In patient 2, the spinal cord was tethered to two slender stalks close to each other, which was visible with the combined use of sagittal and axial postnatal three-dimensional heavily T2-weighted imaging (3D-hT2WI). The preoperative MRI hallmark of saccular LDM is the visualization of a stalk that links the bending cord and sac. Complete untethering surgery to return the cord into the spinal canal and correct its dorsal bending is recommended.