Cervical myelomeningoceles

CERVICAL MYELOMENINGOCELES ARE rare dysraphic lesions. Nine cases of cervical myelomeningoceles are reported. The external features of all nine myelomeningoceles were strikingly similar: They were sturdy, tubular protuberances from the back of the infants' necks, covered at the base by full-thickness skin and covered on the dome by thick squamous epithelium. Internally, these were tethered cord lesions in which fibroneural bands or sagittal midline fibrous septa were tightly tethering the cervical spinal cord to the adjacent dural or intrasaccular soft tissues. Six of our early cases (Group 1) were initially treated with simple subcutaneous resection of the sac and ligation of the dural fistula without release of the internal tethering structures. Five of these children subsequently deteriorated 13 months to 8 years later, all with worsening hand function and spastic legs. All five were reexplored, and the tethering bands and septa were excised; all showed improvement. The other three neonates (Group 2) treated in the last 4 years underwent initial intradural exploration of the lesions; in one case, the tethering fibrous elements were only partially eliminated and the patient deteriorated 4 years later, but improved after a second operation for resection of a missed ventral fibrous septum. The other two Group 2 infants had a thorough release of the fibroneural stalks initially, and both were neurologically stable 3 years later. We recommend that cervical myelomeningoceles should be studied preoperatively with magnetic resonance imaging and computed tomographic myelography to identify the internal structures. The minimum initial surgical treatment should be a two-level laminectomy, intradural exploration, and excision of all tethering bands and septa, in addition to resection of the sac. If a split cord is revealed by imaging studies, both the ventral and dorsal surfaces of the hemicords must be carefully inspected to locate the median septum.