Posterior vertebral column resection for rigid proximal thoracic kyphoscoliosis with broken growing rods in a patient with Desbuquois dysplasia.

Study Design: Case report.
Objective: To describe the importance of preoperative halo-gravity traction and posterior vertebral column resection (PVCR) for severe proximal thoracic kyphoscoliosis associated with Desbuquois dysplasia, after breakage of a growing rod construct. Desbuquois dysplasia is a rare, autosomal recessive chondrodysplasia characterized by short stature, joint laxity, kyphoscoliosis, and characteristic facial dysmorphism. Our 8-year-old patient developed severe, progressive, infantile-onset kyphoscoliosis and had been initially treated with Vertical Expandable Prosthetic Titanium Rib (VEPTR) rods. She subsequently underwent growing rod placement, but the eventual rod fracture resulted in a severe angular kyphosis.
Methods: Clinical and radiographic case review.
Results: The broken implants were removed, and she was treated with 2.5 months of preoperative halo-gravity traction. She then underwent a T4 PVCR and C7-L4 instrumented posterior spinal fusion. The patient had an uneventful postoperative course without any neurologic problems. Two years postoperatively, correction was well maintained with appropriate alignment and balance without implant breakage.
Conclusion: To our knowledge, this is the first report of treatment of spinal deformity associated with Desbuquois dysplasia. Our results suggest that preoperative halo-gravity traction and PVCR are safe and efficacious techniques for severe rigid kyphoscoliosis in the cervicothoracic region associated with broken growing rods in a patient with Desbuquois dysplasia.
Level of Evidence: Level IV.