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. Objectiv es. To determine the most efficacious form of management of spinal def ormities in camptomelic dysplasia. Summary of Background Data. The lit erature 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 functio n. Methods. Eight patients with camptomelic dysplasia and progressive spinal deformity underwent a retrospective chart and radiographic revi ew 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 d egrees kyphosis and 52 degrees scoliosis at follow-up. Complications i ncluded pseudarthrosis (50%) and neurologic problems (33%). Conclusion s. The authors advocate anterior/posterior uninstrumented fusion and h alo cast immobilization postoperatively to prevent curve progression a nd avoid the potentially fatal sequelae associated with this disorder.