Unusual association of congenital kyphosis and conus lipoma presenting as a double spinal cord tether.

The case of a four-year-old child is described who presented to our institution with cervicothoracic deformity and a two-year history of progressive paraparesis. His past medical history was significant for meningocele which was closed at age two months. Imaging studies revealed severe congenital kyphosis with a hypoplastic T3 vertebra, as well as a tethered filum terminale with a conus lipoma. The spinal cord was found to be severely compressed at the apex of the kyphotic deformity. Discussion is focused on the diagnosis of tethered cord syndrome, and treatment options. In particular, this case required careful thought on the order of events, which followed initial tethered cord release and removal of the conus lipoma, and subsequent kyphectomy and fusion of the upper thoracic spine. A favorable clinical outcome was obtained with complete reversal of the paraparesis.