The Centre for Spinal Studies and Surgery, Queens Medical Centre Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom.
The Centre for Spinal Studies and Surgery, Queens Medical Centre Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom.
World Neurosurg. 2019 Nov;131:154-158. doi: 10.1016/j.wneu.2019.07.230. Epub 2019 Aug 6.
Proximal junctional failure (PJF) is a major and sometimes devastating problem following adult spinal deformity (ASD) correction surgery. Common consensus still lags on guidelines for preventing and managing these complications. Surgical treatment of scoliosis in the presence of osteogenesis imperfecta (OI) in the pediatric population is well described. The complication rates are unusually higher in this special subset of patients owing to poor quality of bone. There is a paucity of literature focusing on surgical techniques, strategies, and problems involved in the management of ASD associated with OI.
We report a 59-year-old female with type 1 OI and adult scoliosis who underwent T10-to-pelvis fusion for ASD according to the principles of adult deformity correction. At a 1-year follow-up, she presented with asymptomatic proximal junctional kyphosis of 45° and 2 weeks later had PJF along with spinal cord injury after a fall. On computed tomography scan, kyphosis was increased to 60° at T9-T10. She underwent decompression and revision deformity correction using quadruple rods, with extension of instrumentation to T2 with soft landing using rib bands. At a 4-year follow-up, she had a good functional outcome after revision surgery.
This is the first report of successful management of PJF following ASD correction in the presence of OI using this technique. Suboptimal hold of implants due to poor bone quality must be at the focus of any surgical planning for these patients. All possible strategies to prevent PJF must be considered when planning the deformity correction in adults with OI.
近端交界性失败（PJF）是成人脊柱畸形（ASD）矫正手术后的一个主要问题，有时甚至是灾难性的问题。对于预防和处理这些并发症的指南，目前仍缺乏共识。小儿成骨不全症（OI）患者脊柱侧凸的手术治疗已有详细描述。由于骨质量差，这些特殊患者群体的并发症发生率异常高。目前，关于与 OI 相关的 ASD 的手术技术、策略和管理问题的文献很少。
我们报告了一例 59 岁女性，患有 1 型 OI 和成人脊柱侧凸，根据成人畸形矫正的原则接受了 T10 至骨盆融合术治疗 ASD。在 1 年的随访中，她出现了无症状的近端交界性后凸 45°，2 周后因跌倒导致脊髓损伤并发 PJF。在 CT 扫描上，T9-T10 处的后凸增加到 60°。她接受了减压和矫正畸形的翻修手术，使用四重棒，通过肋骨带将器械延伸至 T2 进行软着陆。在 4 年的随访中，她在翻修手术后功能恢复良好。
这是首例使用该技术成功治疗 OI 合并 ASD 矫正后发生的 PJF 的报告。由于骨质量差，植入物的固定效果不理想，这必须是此类患者任何手术计划的重点。在计划 OI 成人的畸形矫正时，必须考虑所有预防 PJF 的策略。
