• Paul de Baat, Esther van Tankeren, Roderick F A de Lind van Wijngaarden, and Luuk W L de Klerk.
• Department of Paediatric Orthopaedics, Erasmus Medical Centre/Sophia Children's Hospital, Rotterdam, The Netherlands. p.debaat@erasmusmc.nl
• Spine. 2011 Dec 15;36(26):E1765-8.

Study DesignCase report.ObjectiveTo describe a sudden spinal cord injury in a girl with Prader-Willi syndrome (PWS) 1 week after posterior T2-L3 fusion.Summary Of Background DataPWS is a genetic disorder characterized by several features including growth deficiency, hypotonia, obesity, and spinal deformities. In PWS patients the prevalence of scoliosis and of kyphosis is reported to be 15% to 86% and 8% to 40%, respectively. Operative treatment carries specific and serious risks in these patients.MethodsA 13-year-old girl with PWS underwent a ventral release and a posterior spinal fusion for a scoliosis with hyperkyphosis of the thoracic spine. Preoperatively, she had no osteopenia or obesity. Posterior instrumentation consisted of fixation with pedicle screws, proximally assisted by a bilateral transverse hook to prevent junctional hyperkyphosis.ResultsPostoperative recovery was initially uneventful; however, 1 week after operation the patient suddenly suffered a complete spinal cord lesion at level T4. This was due to myelum compression with a spinal dislocation cranial to the fusion level, and subsequent proximal hook failure. The patient underwent immediate removal of the transverse hooks, an extension of the fusion to T1, and a laminectomy at T2. Eighteen months after the event she was still paraplegic.ConclusionThis report describes a sudden and complete spinal cord lesion 1 week after spinal fusion in a child with PWS. Although such an event is often attributed to osteopenia, this was not proven in this case. To our knowledge, this is the first report to describe this event developing so soon and so suddenly after primary surgery. This case report confirms that spinal surgery in patients with PWS carries an increased risk of junctional hyperkyphosis and pullout of hardware.

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