Department of Orthopaedics, The Children's Hospital of Philadelphia, Philadelphia, PA.
Harvard Medical School.
J Pediatr Orthop. 2024;44(10):e929-e937. doi: 10.1097/BPO.0000000000002768. Epub 2024 Jul 11.
Neuromuscular early-onset scoliosis (N-EOS) often presents with a long sweeping thoracolumbar scoliosis and pelvic obliquity. With severe pelvic obliquity, the ribs come into contact with the high side of the pelvis, termed rib-on-pelvis deformity (ROP). The goal of this study is to evaluate whether ROP is associated with reported pain and other health-related quality of life (HRQOL) measures. We hypothesize that ROP is associated with increased pain and negative HRQOL.
A multicenter international registry was queried for all nonambulatory patients with N-EOS from 2012 to 2022. Both surgical and nonsurgical patients were included. ROP was classified as a binary radiographic assessment of preoperative (surgical patients) and most recent follow-up (nonsurgical patients) upright radiographs. Reported pain and other HRQOL measures were assessed through the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24). Patients with nonupright radiographs or EOSQ-24 questionnaires and corresponding radiographs >4 months apart were excluded.
Totally, 225 patients (8.4±3.1 y, 55% female) were included. The median major curve was 63.3 (IQR: 40.6 to 81.2) degrees and median pelvic obliquity was 15.5 degrees (IQR: 8.8 to 26.4). Eighty-three patients (37%) had ROP. ROP was associated with both frequency ( P <0.001) and severity ( P <0.001) of pain. ROP was associated with worse general health ( P =0.01), increased difficulty with vocalization ( P =0.02), increased frequency of shortness of breath ( P =0.002), and increased difficulty sitting upright ( P =0.04). Regarding overall EOSQ-24 domains, ROP was associated with worse general health, pain/discomfort, pulmonary function, and physical function ( P <0.01). In a subanalysis of 76 patients who underwent surgical intervention with at least 2 years of follow-up, patients with preoperative ROP experienced significantly greater improvements in both frequency ( P =0.004) and severity ( P =0.001) of pain than the patients without preoperative ROP at 2 years postoperatively.
The overall incidence of ROP in N-EOS is about 37%. ROP is associated with greater pain and worse HRQOL through the EOSQ-24 questionnaire. Furthermore, these patients experienced a greater reduction in pain after surgery. Clinicians and parents must be aware that ROP is possibly a pain generator, but responds positively to surgical intervention.
Level III.
神经肌肉型早发性脊柱侧凸（N-EOS）常表现为长而广泛的胸腰椎侧凸和骨盆倾斜。骨盆倾斜严重时，肋骨与骨盆高处接触，称为肋骨接触骨盆畸形（ROP）。本研究的目的是评估 ROP 是否与报告的疼痛和其他健康相关生活质量（HRQOL）指标相关。我们假设 ROP 与疼痛增加和 HRQOL 下降有关。
对 2012 年至 2022 年间来自 2 个中心的所有非卧床的 N-EOS 患者进行了多中心国际登记。包括手术和非手术患者。ROP 通过术前（手术患者）和最近随访（非手术患者）的直立位 X 线片进行影像学评估。通过 24 项早发性脊柱侧凸问卷（EOSQ-24）评估报告的疼痛和其他 HRQOL 指标。排除无直立位 X 线片或 EOSQ-24 问卷且相应 X 线片与 X 线片之间间隔超过 4 个月的患者。
共纳入 225 例患者（8.4±3.1 岁，55%为女性）。主要弯曲中位数为 63.3（IQR：40.6-81.2）度，骨盆倾斜中位数为 15.5 度（IQR：8.8-26.4）。83 例（37%）患者存在 ROP。ROP 与疼痛的频率（ P <0.001）和严重程度（ P <0.001）相关。ROP 与一般健康状况恶化（ P =0.01）、发声困难（ P =0.02）、呼吸急促频率增加（ P =0.002）和难以坐直（ P =0.04）有关。在接受至少 2 年随访的 76 例手术干预患者的亚分析中，术前存在 ROP 的患者在术后 2 年时，疼痛频率（ P =0.004）和严重程度（ P =0.001）的改善均显著大于术前无 ROP 的患者。
N-EOS 中 ROP 的总体发生率约为 37%。ROP 通过 EOSQ-24 问卷与更大的疼痛和更差的 HRQOL 相关。此外，这些患者在手术后疼痛减轻更明显。临床医生和家长必须意识到，ROP 可能是疼痛的一个来源，但对手术干预反应良好。
III 级。
