Posterior vertebral column resection for VATER/VACTERL associated spinal deformity: a case report

Abstract

The VATER/VACTERL association is a syndrome notable for congenital vertebral malformations, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, and renal or limb malformations. Vertebral malformations may include the entire spectrum of congenital spinal deformities, including kyphosis, as was seen in this case. A 14-year-old girl presented to our institution with severe rigid sagittal deformity in the thoracolumbar spine that had recurred following three prior spinal fusion surgeries: the first posterior only, the second anterior and posterior, and the third a posterior only proximal extension. These surgeries were performed to control progressive kyphosis from a complex failure of segmentation that resulted in a 66 degrees kyphosis from T11 to L3 by the time she was 9 years old. Our evaluation revealed solid arthrodesis from the most recent procedures with resultant sagittal imbalance, and surgical options to restore balance included anterior and posterior revision spinal fusion with osteotomies, multiple posterior extension osteotomies with circumferential spine fusion, and posterior vertebral column resection with circumferential spine fusion. She was advised that multiple posterior extension osteotomies would likely be insufficient to restore sagittal balance in the setting of solid arthrodesis from anterior and posterior surgery, and that the posterior-only vertebral column resection would provide results equivalent to revision anterior and posterior surgery, without the morbidity of the anterior approach. She successfully underwent posterior vertebrectomy and circumferential spinal fusion with instrumentation and is doing well 2 years postoperatively. Severe rigid sagittal deformity can be effectively managed with a posterior-only surgical approach, vertebrectomy, and circumferential spinal fusion with instrumentation.

Fig. 1

(a) Preoperative full-length lateral x-ray showing prior single rod posterior instrumentation from T3–L5, kyphosis from T8–L4 measuring 87° by Cobb method, and gibbus deformity at L1–L2. (b) Postoperative full-length lateral X-ray showing improved sagittal balance and kyphosis from T8–L4 measuring 46 degrees by Cobb method. (c) Preoperative clinical photograph demonstrating thoracolumbar kyphosis with high lumbar gibbus deformity, and poor sagittal alignment. (d) Postoperative clinical photograph showing improved sagittal contour and balance

Fig. 2

Schematic diagrams illustrating the surgical technique of posterior vertebral column resection. (a) Old instrumentation was used for temporary fixation of the spine while additional pedicle screws were added, posterior elements were removed at the apex of the deformity, and laminectomies were performed. Note the increased space available for the spinal cord and dural sac (stippled structure in the diagram). (b) After rib head removal, high-speed burr was used to remove the vertebral bodies. (c) The old rod was then removed, new rods were placed proximally and distally, Harms cage was placed anteriorly, and cantilever maneuver was used to reduce the spinal segments. (d) Proximal and distal instrumentation was then connected and tightened, and bone graft was placed around the Harms cage and within the intended fusion bed