Double spinal cord tethering and congenital kyphosis in a 4-year-old boy

Purpose Congenital kyphosis (CK) is an uncommon condition that develops due to a defect of formation or segmentation of one or more vertebrae during the first weeks of embryonic life and can be frequently associated with abnormalities of the spinal cord. Meningocele manque (MM) is a rare congenital malformation consisting of intradural fibrous bands tethering the spinal cord, oftentimes in combination with a split cord malformation. The aim of this manuscript is to describe combined surgical management of a rare case of CK associated with double spinal cord tethering. Study design Case report. Methods We report the case of a 4-year-old boy presenting with a combination of congenital kyphosis due to L2 fully segmented posterior hemivertebra and double spinal cord tethering, at the level of the filum terminale and at L1-L2 due to an intradural ventral fibrous band. Results Spinal cord detethering and L2 hemivertebra resection with kyphosis correction were achieved with a single stage procedure. Segmental kyphosis was corrected from 45 degrees to 11 degrees post-operatively. Apart from a transitory sensory impairment, no immediate or delayed complications were noted and, at 2 years follow-up, correction and spinal alignment were stable. Conclusion To the best of our knowledge, this is the first case to report the simultaneous occurrence of congenital kyphosis and double spinal cord tethering due to MM and tethering of the filum terminale. Our case report shows that surgical correction of the deformity and spinal cord detethering can be safely and effectively achieved during the same surgical procedure.