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Scoliosis associated with Prader-Willi syndrome
Kazunari Yamada1, Kei Miyamoto, Hideo Hosoe
1Department of Orthopaedic Surgery, Gifu University, Graduate School of Medicine, Yanagido 1-1, Gifu City, Gifu 501-1194, Japan.
Background Context:
Prader-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.
Purpose:
To 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/Setting:
Case report of a patient treated in Gifu, Japan.
Methods:
The patient, a 16-year-old girl with Prader-Willi syndrome, had severe scoliosis with triple curves (T1 to T5, 43 degrees T5 to T11, 60 degrees; T11 to L3, 52 degrees), 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.
Results:
The thoracic curve was corrected to 21 degrees (correction rate, 65%) and the lumbar curve to 28 degrees (correction rate, 46%). Her symptoms were relieved.
Conclusion:
A 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.
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