Lumbosacral kyphosis, tethered cord, and diplomyelia. A unique spinal dysraphic condition

Four patients are described with a triad of neuroectodermal abnormalities consisting of lumbosacral kyphosis, tethered cord, and dyplomyelia. Of utmost importance is the recognition of the association between this type of kyphosis and the underlying spinal cord pathology and the progressive nature of the deformity. The patient presenting with lumbosacral kyphos and the presence of sacral hypoplasia should have a neuroradiographic evaluation (magnetic resonance imaging scanning or enhanced computed tomography) to rule out the presence of a tethered cord or other neural abnormalities. Early surgical intervention for release of the tether and fusion should be carried out to prevent neurologic deterioration and curve progression.