Scoliosis associated with Prader-Willi syndrome

Background contextPrader-Willi syndrome is a rare disease associated with a variety of musculoskeletal abnormalities, including scoliosis, joint hyperlaxity, and delayed bone age. To the authors' knowledge, only a few cases of surgical treatment for scoliosis associated with Prader-Willi syndrome have been reported.PurposeTo report a rare case of scoliosis associated with Prader-Willi syndrome and the effect of surgical treatment and to review the literature on this condition.Study design/settingCase report of a patient treated in Gifu, Japan.MethodsThe patient, a 16-year-old girl with Prader-Willi syndrome, had severe scoliosis with triple curves (T1 to T5, 43° T5 to T11, 60°; T11 to L3, 52°), making it difficult for her to maintain balance while standing or walking. She underwent surgical correction and fusion for the scoliosis via the posterior approach. She was followed up for 2 years, and her clinical symptoms and plain X-ray films were evaluated.ResultsThe thoracic curve was corrected to 21° (correction rate, 65%) and the lumbar curve to 28° (correction rate, 46%). Her symptoms were relieved.ConclusionA case of scoliosis with Prader-Willi syndrome was successfully treated surgically using a posterior approach and minimizing possible risks associated with surgery in patients with this syndrome.