Long-term outcome for patients with split cord malformation

Abstract

Split cord malformations (SCMs) are relatively rare forms of occult spinal dysraphism (OSD) and tethered spinal cord syndrome. The majority of these cases present in early childhood, with neurocutaneous stigmata being an early presenting feature. Prophylactic detethering surgery is advocated by most neurosurgeons due to the risk of neurological deterioration over time caused by patient growth and activity. However, unlike other forms of OSD, the course of SCM progression after surgery is not well understood, and little has been published about long-term followup results. In this study the authors review the results obtained in 16 patients in whom the senior author performed surgery over a 13-year period (average length of follow up almost 8 years). Presentation, surgical approach, and outcome are evaluated, and the long-term outcome of neurological status, pain, bowel/bladder disturbance, and spinal deformities are emphasized. The primary conclusion is that patients with SCM generally tolerate surgery well and experience few complications. Neurological deterioration is rare except in cases in which retethering occurs, (two patients in this series). Although impaired bowel and bladder function was stabilized or improved and pain was reliably relieved postoperatively, preexisting vertebral column deformities usually progressed after surgery and, in most cases, required spinal fusion.