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Virtual Pediatric Hospital: Paediapaedia: Diastematomyelia Paediapaedia: Neurological Diseases

Diastematomyelia

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Have neurological deficits in the lower limbs and sphincteric disturbances such as gait disturbances, muscular atrophy, reflex changes, and foot deformities which do not manifest themselves until walking begins.

Etiology/Pathophysiology:
Part of the spinal dysraphism complex. Is a congenital longitudinal split of the spinal cord or filum terminale. The etiology unclear, and perhaps it is an attempted spinal canal duplication. Can be associated with intraspinal neuroenteric cysts, dermoid cysts, teratomas, and lipomas. It is the cause of 5% of congenital scoliosis.

Pathology:
Not applicable

Imaging Findings:
The cord is widened and split into 2 lateral halves between which lies the longitudinal septum composed of fibrous tissue and / or cartilage and / or a small spicule of bone. The dividing septum is attached anteriorly to the ventral wall of the spinal canal and posteriorly to the dura and can extend for several vertebral bodies. The septum fixes the spinal cord at the involved level so the cord cannot make its normal shift cephalad as the vertebral column lengthens. Can extend for several vertebral bodies. The cord usually is divided asymmetrically and then reunites below the cleft.

CT defines the nature of the spicule, whether it is bony or not.

DDX:

References:
See References Chapter.

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