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


METHODS
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.


CONCLUSIONS
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.
Muroi
Ai
A
Division of Neurosurgery, Childrens Hospital Los Angeles, Suite 1006, Los Angeles, CA 90027, USA. muroiai@aol.com
Fleming
Karen L
KL
McComb
J Gordon
JG
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007714:Klippel-Feil Syndrome / Q000473:pathology; D008279:Magnetic Resonance Imaging; D009333:Neck / Q000002:abnormalities; D009436:Neural Tube Defects / Q000473:pathology*; D009777:Occipital Bone / Q000002:abnormalities; D016136:Spina Bifida Occulta / Q000473:pathology; D013116:Spinal Cord / Q000002:abnormalities* / Q000473:pathology*; D013118:Spinal Cord Diseases / Q000151:congenital / Q000473:pathology*; D013131:Spine / Q000473:pathology; D014057:Tomography, X-Ray Computed