Prader-Willi Syndrome (PWS) is a chromosome 15 disorder characterized by hypotonia, hypogonadism, hyperphagia, and obesity. Musculoskeletal manifestations, including scoliosis, are well described. For prevention of progression of scoliosis with PWS, brace treatment alone is not enough. Therapeutic options in scoliosis with PWS are limited by many complications associated with surgery. Here we report a case of scoliosis with PWS treated with anterior spinal fusion.
Case: A male, aged 15 years, diagnosed with PWS at birth. He had overt progression of thoracolumbar scoliosis with double major curves of 30 degrees and 60 degrees. Anterior spinal fusion with double holed vertebral staples was performed. Transient atelectasis occurred after the surgery, but there were no other severe complications. One year and 8 months after surgery, the curves were held at 18 degrees and 15 degrees.
Discussion: Traditionally, operative scoliosis with PWS has been treated with a posterior spinal fusion. Anterior spinal fusion has an advantage of solid fixation and short spinal fusion at once, compared to posterior spinal fusion. Furthermore, in this case, double holed vertebral staples were used and 2 screws were inserted in each vertebral body to tighten the fixation of instrumentation on breakable vertebrae with PWS. As a result, better correction of the scoliosis was obtained in this case than that in reports of posterior spinal fusions. There was little loss of spinal fixation and correction of scoliosis within for 1 year and 8 month after surgery. An anterior spinal fussion can be a particularly effective surgery for scoliosis with PWS.
