The treatment of progressive kyphoscoliosis in camptomelic dysplasia.

STUDY DESIGN: This study evaluated the different forms of treatment of camptomelic dysplasia, a rare form of short-limbed dwarfism.

OBJECTIVES: To determine the most efficacious form of management of spinal deformities in camptomelic dysplasia.

SUMMARY OF BACKGROUND DATA: The literature on treatment of spinal deformities in camptomelic dysplasia is sparse. One report advocates aggressive surgical treatment to prevent curve progression and prevent already compromised respiratory function.

METHODS: Eight patients with camptomelic dysplasia and progressive spinal deformity underwent a retrospective chart and radiographic review by an independent observer. Follow-up averaged 3 years and 9 months.

RESULTS: Five of eight patients initially were treated with bracing and six of eight patients eventually required surgery. Average initial kyphosis was 114 degrees and scoliosis 61 degrees, compared with 99 degrees kyphosis and 52 degrees scoliosis at follow-up. Complications included pseudarthrosis (50%) and neurologic problems (33%).

CONCLUSIONS: The authors advocate anterior/posterior uninstrumented fusion and halo cast immobilization postoperatively to prevent curve progression and avoid the potentially fatal sequelae associated with this disorder.