Split cord malformations

Abstract

Previous classifications of split cord malformations (SCM's), including such terms as diastematomyelia and diplomyelia, have done little to foster a true understanding of these disorders. The authors instead propose a unifying classification in which all SCM's share a common embryogenesis. SCM's are divided into two types, based upon the composition of the dural coverings and intervening mesenchymal tissue. Type I malformations are composed of two dural sacs and a bony or fibrocartilaginous spur; Type II malformations are composed of a single dural sac and intradural fibrous bands. In either case, the intervening mesenchymal elements contribute to progressive neurologic, urologic, and orthopedic deterioration from spinal cord tethering. The natural history of these lesions supports an early and aggressive operative approach to untether the spinal cord before clinical deterioration begins.