Vertebral column resection for complex congenital kyphoscoliosis and type I split spinal cord malformation

Congenital progressive kyphoscoliosis associated with split spinal cord malformation (SSCM) is a very rare disease.

Here, we present the case of a 23-year-old woman who was found kyphoscoliosis when she was 10 years old and developed rapidly. Thereafter, no management was proposed before her consultation at our center. On examination, numbness was found in the right low limbs, kyphoscoliosis was detected in thoracolumbar, the trunk deviated to the right on standing view and curvature of the thoracolumbar spine was left-sided, the left rib hump was severe and there was little muscle tissue felt under the right side paravertebral area, the pelvis was oblique with the right side higher than the left side, the right arcus costarum was 5 cm below the right iliac crest. One-stage corrective surgery was determined, at first, halo-wheelchair traction gradually with increased traction weights out of hospital for a month; then, after a reasonable correction achieved without any neurological deficits. The one-stage operation was taken through single posterior segmental pedicle screw instrumented fusion with VCR between T12 and L1. Post-operative recovery was uneventful and there were no complications, she was discharged 10 days post operation. At 2 years follow-up, the patient’s outcome is excellent with balance and correction of the deformity.

Based on the Grand Round case and relevant literature, we discuss the different options for the treatment of congenital kyphoscoliosis associated with type I SSCM. In the patient whose kyphoscoliosis is severe and rigid, we recommend an initial release followed by halo-wheelchair traction gradually to correct the deformity, once optimal correction acquires during the traction, the posterior instrumented fusion with VCR upper the bony spur could be done safely without the resection of bony spur.