Split medulla in association with multiple closed neural tube defects

We describe the case of a patient with a split medulla associated with multiple closed neural tube defects.

This 13-year-old female initially presented to our service at just over a year of age for neck abnormalities and a congenital midline occipital dermal sinus. MRI and CT scans showed the presence of multiple cervical vertebral segmentation abnormalities, a bony spur at the level of the craniocervical junction, split medulla, split cervical spinal cord, and a midline occipital dermal sinus. Since the patient's neurological course had remained stable during follow-up for over 11 years, the only surgical procedure undertaken was excision of the dermal sinus to prevent infection.

The constellation of abnormalities indicates that the notochord was split early during gestation, both rostral and caudal to the cervicomedullary junction. Surgical intervention to treat the other malformations needs to be considered only if the patient develops a progressive neurological deficit.