• Franck Accadbled, Thierry Odent, Alexandre Moine, Edouard Chau, Christophe Glorion, Gwenaelle Diene, and Jérôme Sales de Gauzy.
• Department of Orthopedic Surgery, Hôpital des Enfants, Toulouse, France. faccadbled@wanadoo.fr
• Spine. 2008 Feb 15;33(4):394-401.

Study DesignRetrospective case series of 16 consecutive patients.ObjectiveTo evaluate the results of scoliosis surgery in Prader-Willi syndrome (PWS) with special emphasis on the complications and their risk factors.Summary Of Background DataPWS is a rare genetic disorder characterized by obesity, hypotonia, and frequent scoliosis. The literature dealing with scoliosis surgery in PWS consists of only few case reports. Surgical treatment was reported to be difficult with frequent and severe complications.MethodsSixteen patients (3 males, 13 females) in 63 presenting scoliosis and PWS were operated on between 1974 and 2004. Preoperative, postoperative and last follow-up clinical and radiologic data were analyzed. Complications, treatment, and outcome were investigated.ResultsMean age at scoliosis diagnosis was 6.2 years (range 0.5-13.5). Mean age at surgery was 12.3 years (range 5-15). Mean follow-up was 5.4 years (range 2-18). There were 9 major complications (4 severe kyphosis above fusion, 2 deep infections, 1 transient paraplegia, 1 pseudarthrosis, 1 delayed wound healing). The 4 kyphosis required reoperation, 3 of which were complicated with permanent spinal cord injury. Minor complications affected 6 patients.ConclusionScoliosis surgery is frequently necessary in PWS and is associated with high rate of complications. These are often related to specific features of this syndrome the surgeon should recognize and consider.

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