Shriners Hospital for Children, Philadelphia, PA.
J Pediatr Orthop. 2021 Apr 1;41(4):e321-e327. doi: 10.1097/BPO.0000000000001660.
Prader-Willi syndrome (PWS) is a rare genetic syndrome, with a prevalence of infantile scoliosis of ~23%. These curves are likely related to severe hypotonia. Approximately 15% of children with PWS will need surgical intervention for their scoliosis. The purpose of this study was to evaluate the effectiveness of curing or controlling moderate and severe infantile scoliosis curves in children with PWS.
This single institution, retrospective study of patients with PWS and infantile scoliosis reviewed 34 consecutive children with >24 months follow-up from initiation of serial spinal casting. Cobb angle comparison measurements of radiographs taken precasting, during treatment, and at follow-up were performed. Rib-vertebral angle difference, Nash-Moe rotation, and space available for lung measurements were followed. Outcomes were stratified as "Cured," "Braced," and "Surgery."
Average age for first cast for the entire study was 32 months (range, 14 to 64), undergoing 8 casts (range, 3 to 18) over 25 months (range, 9 to 57) for an initial curve of 54 degrees (range, 27 to 106 degrees), which improved to 27 degrees (range, 11 to 78 degrees). In total, 12 patients (35%) were in the Cured group, following 6 casts over 17 months, with an initial curve of 44±14 degrees improving to 17±5 degrees at the end of treatment, and 20±18 degrees at 68-month follow-up. In total, 18 patients were in the Braced group, with curves initially improving from 55±14 degrees to 35±14 degrees, but at 47±20 degrees at 51-month follow-up. Four patients needed surgery, with initial curves 85 degrees (range, 54 to 106 degrees), but surgery could be postponed 56 months (range, 40 to 73) by casting. Rib-vertebral angle difference was not prognostic.
Serial spinal casting is effective in for treating infantile scoliosis in children with PWS. One third of patients had their curve resolved, at least temporarily, where they were braced and cast free. The others were able to delay surgery for a number of years. Initial curves <50 degrees in children <3 years of age seem to have the best prognosis.
Level IV.
普拉德-威利综合征（PWS）是一种罕见的遗传综合征，婴儿型脊柱侧凸的患病率约为 23%。这些曲线可能与严重的低张力有关。大约 15%的 PWS 患儿需要手术干预其脊柱侧凸。本研究的目的是评估治疗或控制 PWS 患儿中中度和重度婴儿型脊柱侧凸曲线的效果。
这项回顾性研究纳入了 34 例 PWS 合并婴儿型脊柱侧凸的患者，这些患者在开始连续脊柱铸型治疗后，有超过 24 个月的随访。对铸型前、治疗过程中以及随访时拍摄的 X 线片进行 Cobb 角比较测量。同时还进行了肋骨-椎体角差、Nash-Moe 旋转和肺间隙测量。结果分为“治愈”“支具治疗”和“手术”。
整个研究中，所有患者首次接受铸型的平均年龄为 32 个月（1464 个月），接受 8 次铸型治疗（318 次），持续 25 个月（957 个月），初始弯曲度为 54 度（27106 度），改善至 27 度（1178 度）。总共有 12 例（35%）患者归入“治愈”组，接受了 6 次铸型治疗，治疗 17 个月，初始弯曲度为 44±14 度，治疗结束时改善至 17±5 度，68 个月随访时改善至 20±18 度。总共有 18 例患者归入“支具治疗”组，弯曲度最初从 55±14 度改善至 35±14 度，但在 51 个月随访时，仍有 47±20 度。4 例患者需要手术治疗，初始弯曲度为 85 度（54106 度），但通过铸型治疗可以将手术推迟 56 个月（40~73 个月）。肋骨-椎体角差无预后价值。
连续脊柱铸型治疗对 PWS 患儿的婴儿型脊柱侧凸有效。三分之一的患者的弯曲度至少暂时得到缓解，无需支具和铸型治疗。其余患者的手术也可推迟数年。3 岁以下、初始弯曲度<50 度的患儿似乎具有最佳的预后。
IV 级。
