Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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August 13, 2012
Diastematomyelia with Intramedullary Dermoid
- Diastematomyelia is an uncommon type of spinal dysraphism in which there is longitudinal clefting of the cord.
- Two types have been described: Type I, in which the 2 hemicords are contained in a single dural envelope, and type II, in which the 2 hemicords are contained in their own separate dura.
- The onset of symptoms can be seen any time from birth to adult life. Among newborns the presence of an abnormal tuft of hair over the lower spine or an associated meningocele brings the defect to attention. Neurologic deficits include foot deformities, muscular atrophy, extremity shortening, bowel bladder incontinence, paresthesias, reflex abnormalities and gait disturbances.
- Key Diagnostic Features: Two hemicords separated by a fibrous/bony spur with/without syringohydromyelia. Associated findings include segmentation anomalies, myelomeningocele, tethered cord, and, rarely, dermoid/epidermoid. Rarely, diplomyelia (duplicated spinal cord) is seen.
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