Systematic Review of Urologic Outcomes from Tethered Cord Release in Occult Spinal Dysraphism in Children

Tethered cord syndrome describes a condition of multisystem end organ dysfunction due to fixation of the spinal cord. This systematic review focuses on the closed skin variant of this condition, occult spinal dysraphism. The embryology, pathophysiology, presentation, and classification of occult spinal dysraphism are explained to develop a simple framework for discussions regarding this often confusing condition. Following Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines, we synthesized urologic outcome data after tethered cord release in children from 17 studies performed over the past 25 years. These results prompted several conclusions. First, the different subgroups and different nomenclature of tethered cord syndrome are often confused, making interpretation of results difficult. Second, untethering has a positive effect on urologic symptoms and urodynamics parameters. Third, timing of untethering is important: early intervention prevents significant long-term traction aiming to avoid irreversible neurologic damage. Fourth, pediatric urologists and neurosurgeons have an important role in diagnosing and treating this condition and should work closely as part of a multidisciplinary team.