Spinal Column Shortening for Secondary Tethered Cord Syndrome in Children: 2-Dimensional Operative Video

Tethered cord syndrome is a constellation of signs and symptoms involving sensory and motor dysfunction of the lower extremities, bladder, and bowel. The pathophysiology may be from a mechanical stretch of the distal spinal cord causing deleterious changes in blood supply and metabolism. The standard of care for tethered cord syndrome has been tethered cord release. However, drawbacks of traditional tethered cord release include neurological injury, cerebrospinal fluid leak, and intradural scarring and retethering of the spinal cord (secondary tethering). As such, spinal column shortening was described to avoid these risks.1 We applied spinal column shortening to children and transitional adults with secondary tethered cord syndrome.1 As shown in cadaveric biomechanical studies, the objective of surgery is to shorten by the spinal column by 1.5 to 2.5 cm, to obtain the equivalent relief of traction on the spinal cord and nerve roots as direct circumferential untethering.2 In this video, we present the case of an 11-yr-old boy with a history of VATERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) and tethered cord syndromes manifest as bladder dysfunction. Our patient had segmentation and formation anomalies of the lumbosacral junction, imperforate anus, and leg length discrepancy. He had already undergone multiple tethered cord releases with no durable improvement in bladder function. After discussion of the advantages and disadvantages of spinal column shortening with the patient and his father, we decided to proceed with this treatment alternative. At 9 mo after surgery, the patient has satisfactory clinical and radiographic outcomes. Informed consent was obtained from the parents of the patient.