Pagadala Manasa, Marx Jeremy, Brecount Hogan, Carney John, Gerlach Erik, Swiatek Peter, Sarwark John
Department of Orthopaedic Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, 60611, USA.
J Orthop. 2023 Feb 17;37:75-80. doi: 10.1016/j.jor.2023.02.004. eCollection 2023 Mar.
The prevalence and treatment of severe scoliosis and other spinal anomalies in patients with Turner's syndrome (TS) is not well reported. This is the largest case series to date regarding the treatment course and outcomes of severely scoliotic TS patients.
A retrospective chart review was performed to identify all patients with TS seen at a single center academic pediatric institution from 2007 to 2021. Of these, the presence of concomitant severe scoliosis or other spinal anomalies was determined, defined by a major coronal curve measuring 45° or greater. Demographic, clinical, surgical, and radiologic data was collected at both pre- and post-intervention time points.
A retrospective chart review identified 306 patients with TS. Of those, six were identified to have severe scoliosis or other severe spinal anomalies requiring fusion. All four posterior spinal fusion (PSF) patients demonstrated improvement of their spinal curvature. One patient who electively pursued only bracing demonstrated minimal improvement and surgery was subsequently recommended, but not pursued. One patient expired from a pre-existing heart condition prior to intervention. All postoperative complications resolved with no further complications. The only brace-related complication was an allergic rash related to the brace material.
All four patients who underwent PSF demonstrated significant improvement of their spinal curvature with few post-surgical complications. None of the patients in the bracing cohort demonstrated stabilization of their spinal curvature. Therefore, these data corroborate with prior studies, suggesting that operative management consisting of spinal fusion with instrumentation provides optimal clinical outcomes, compared to bracing only.
关于特纳综合征（TS）患者中重度脊柱侧弯及其他脊柱异常的患病率和治疗情况，相关报道并不充分。这是迄今为止关于重度脊柱侧弯TS患者治疗过程和结果的最大病例系列。
进行了一项回顾性病历审查，以确定2007年至2021年在一家单一中心学术儿科机构就诊的所有TS患者。其中，确定是否存在合并的重度脊柱侧弯或其他脊柱异常，定义为冠状面主弯角度测量为45°或更大。在干预前和干预后的时间点收集人口统计学、临床、手术和放射学数据。
回顾性病历审查确定了306例TS患者。其中，6例被确定患有重度脊柱侧弯或其他需要融合的严重脊柱异常。所有4例接受后路脊柱融合术（PSF）的患者脊柱侧弯均有改善。1例仅选择支具治疗的患者改善甚微，随后建议手术，但患者未接受。1例患者在干预前因原有心脏病死亡。所有术后并发症均已解决，未出现进一步并发症。唯一与支具相关的并发症是与支具材料有关的过敏性皮疹。
所有接受PSF的4例患者脊柱侧弯均有显著改善，术后并发症较少。支具治疗组的患者脊柱侧弯均未得到稳定。因此，这些数据与先前的研究一致，表明与仅使用支具相比，采用器械辅助脊柱融合的手术治疗可提供最佳临床结果。
