Oore Jonathan, Connell Braydon, Yaszay Burt, Samdani Amer, Hilaire Tricia St, Flynn Tara, El-Hawary Ron
IWK Health Centre.
Dalhousie University, Halifax, NS, Canada.
J Pediatr Orthop. 2019 Sep;39(8):e597-e601. doi: 10.1097/BPO.0000000000001123.
Prader-Willi syndrome (PWS) patients can present with scoliosis which can be treated with serial cast correction (SCC) or with growth friendly surgery (GFS). This study's purpose was to describe the results of SCC as well as GFS for PWS patients with early-onset scoliosis (EOS).
PWS patients were identified from 2 international multicenter EOS databases. Scoliosis, kyphosis, spine height (T1-S1), right/left hemithoracic heights/widths (RHTH, LHTH, RHTW, LHTW) were measured pretreatment, postoperation, and at 2-year follow-up. Complications were recorded.
Overall, 23 patients with 2-year follow-up were identified. Pretreatment; patients treated with SCC (n=10) had mean age of 1.8±0.6 years; body mass index (BMI), 16±1.5 kg/m; scoliosis, 45±18 degrees; kyphosis, 56±9 degrees; T1-S1, 22.4±2.4 cm; RHTH, 8.0±2.0 cm; LHTH, 8.5±1.7 cm; RHTW, 6.6±1.3 cm; and LHTW, 8.0±1.0 cm. Patients treated with GFS (n=13) had mean age of 5.8±2.6 years; BMI, 21±5.4 kg/m; scoliosis, 76±14 degrees; kyphosis, 59±25 degrees; T1-S1, 24.1±3.6 cm; RHTH, 10.0±1.6 cm; LHTH, 10.6±1.6 cm; RHTW, 9.4±2.5 cm; and LHTW, 8.1±2.8 cm. At 2-year follow-up, patients treated with SCC had mean scoliosis 37±11 degrees (18% correction, P=0.06); kyphosis, 42±6 degrees (NS); T1-S1, 26.4±2.1 cm (P<0.01); RHTH, 9.0±1.1 cm (13%; P=0.30); LHTH, 10.0±1.5 cm (18%, P<0.01); RHTW, 7.4±1.1 cm (12%, P<0.01); and LHTW, 8.0±1.0 cm (0%, P=0.34). At 2-year follow-up, patients treated with GFS had mean scoliosis 42±13 degrees (45% correction, P<0.000001); kyphosis, 53±13 degrees (10%, P=0.19); T1-S1, 31.5±5.4 cm (P<0.00001); RHTH, 12.0±2.4 cm (20%; P<0.01); LHTH, 12.0±1.7 cm (13%; P<0.01); RHTW, 9.8±1.3 cm (4%; P=0.27); and LHTW, 7.9±2.3 cm (3%;P=0.11). As an entire group, patients with a BMI>17 kg/m² had more device-related than disease-related complications (P=0.09). Patients treated with SCC had 0.9 complications per patient. Patients treated with GFS had 2.2 complications per patient [≤5 y more often had ≥2 complications (P=0.05)].
At 2-year follow-up, SCC and GFS were both effective in treating EOS in PWS patients. Patients treated with SCC had significant improvements in spine height and LHTH. Patients treated with GFS had significant improvements in scoliosis magnitude, spine height, RHTH, and LHTH.
Level IV-therapeutic study.
普拉德-威利综合征（PWS）患者可出现脊柱侧弯，可采用系列石膏矫正（SCC）或生长友好型手术（GFS）进行治疗。本研究的目的是描述SCC以及GFS治疗早发性脊柱侧弯（EOS）的PWS患者的结果。
从2个国际多中心EOS数据库中识别出PWS患者。在术前、术后及2年随访时测量脊柱侧弯、后凸、脊柱高度（T1-S1）、右/左半胸高度/宽度（RHTH、LHTH、RHTW、LHTW）。记录并发症情况。
总体而言，共识别出23例有2年随访资料的患者。术前，接受SCC治疗的患者（n = 10）平均年龄为1.8±0.6岁；体重指数（BMI）为16±1.5kg/m；脊柱侧弯为45±18度；后凸为56±9度；T1-S1为22.4±2.4cm；RHTH为8.0±2.0cm；LHTH为8.5±1.7cm；RHTW为6.6±1.3cm；LHTW为8.0±1.0cm。接受GFS治疗的患者（n = 13）平均年龄为5.8±2.6岁；BMI为21±5.4kg/m；脊柱侧弯为76±14度；后凸为59±25度；T1-S1为24.1±3.6cm；RHTH为10.0±1.6cm；LHTH为10.6±1.6cm；RHTW为9.4±2.5cm；LHTW为8.1±2.8cm。在2年随访时，接受SCC治疗的患者脊柱侧弯平均为37±11度（矫正18%，P = 0.06）；后凸为42±6度（无显著性差异）；T1-S1为26.4±2.1cm（P<0.01）；RHTH为9.0±1.1cm（13%；P = 0.30）；LHTH为10.0±1.5cm（18%，P<0.01）；RHTW为7.4±1.1cm（12%，P<0.01）；LHTW为8.0±1.0cm（0%，P = 0.34）。在2年随访时，接受GFS治疗的患者脊柱侧弯平均为42±13度（矫正45%，P<0.000001）；后凸为53±13度（10%，P = 0.19）；T1-S1为31.5±5.4cm（P<0.00001）；RHTH为12.0±2.4cm（20%；P<0.01）；LHTH为12.0±1.7cm（13%；P<0.01）；RHTW为9.8±1.3cm（4%；P = 0.27）；LHTW为7.9±2.3cm（3%；P = 0.11）。作为一个整体组，BMI>17kg/m²的患者与器械相关的并发症多于与疾病相关的并发症（P = 0.09）。接受SCC治疗的患者人均有0.9次并发症。接受GFS治疗的患者人均有2.2次并发症[≤5岁的患者更常出现≥2次并发症（P = 0.05）]。
在2年随访时，SCC和GFS对治疗PWS患者的EOS均有效。接受SCC治疗的患者脊柱高度和LHTH有显著改善。接受GFS治疗的患者脊柱侧弯程度、脊柱高度、RHTH和LHTH有显著改善。
IV级治疗性研究。
