Department of Paediatric Orthopaedics, Erasmus Medical Centre/Sophia Children's Hospital, Rotterdam, The Netherlands.
Spine (Phila Pa 1976). 2011 Dec 15;36(26):E1765-8. doi: 10.1097/BRS.0b013e318216d357.
Case report.
To describe a sudden spinal cord injury in a girl with Prader-Willi syndrome (PWS) 1 week after posterior T2-L3 fusion.
PWS 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.
A 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.
Postoperative 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.
This 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.
病例报告。
描述一名患有普拉德-威利综合征（PWS）的女孩在 T2-L3 后路融合术后 1 周突发脊髓损伤。
PWS 是一种遗传性疾病，其特征包括生长发育迟缓、低张力、肥胖和脊柱畸形。在 PWS 患者中，脊柱侧凸和后凸的患病率分别为 15%至 86%和 8%至 40%。在这些患者中，手术治疗存在特定的严重风险。
一名 13 岁的女孩患有 PWS，因胸椎侧凸伴过度后凸行前路松解和后路脊柱融合术。术前她没有骨质疏松症或肥胖症。后路器械固定采用椎弓根螺钉固定，近端通过双侧横钩辅助，以防止交界性后凸。
术后恢复起初顺利；然而，术后 1 周，患者突然出现 T4 水平完全性脊髓损伤。这是由于融合水平以上的脊髓脱位导致脊髓受压，随后横钩失败。患者立即行横钩切除、融合延长至 T1 和 T2 椎板切除术。事件发生后 18 个月，她仍截瘫。
本报告描述了一名患有 PWS 的儿童在脊柱融合术后 1 周突发完全性脊髓损伤。尽管这种情况通常归因于骨质疏松症，但在本例中并未得到证实。据我们所知，这是首例描述这种事件在初次手术后如此迅速和突然发生的报告。本病例报告证实，PWS 患者的脊柱手术存在交界性后凸和内固定物拔出的风险增加。
