Omara Chady, Pieters Lynn, Castelein René M, Sakkers Ralph J B, Vleggeert-Lankamp Carmen L A
Department of Neurosurgery, Leiden University Medical Center (LUMC), Albinusdreef 2, Leiden, 2333 ZA, The Netherlands.
Department of Neurosurgery, Computational Neurosciences Outcome Center (CNOC), Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
Eur Spine J. 2025 Apr;34(4):1221-1228. doi: 10.1007/s00586-025-08692-5. Epub 2025 Feb 3.
Thoracolumbar kyphosis (TLK) is frequently reported in children with achondroplasia. The combination of TLK and the narrow spinal canal in achondroplasia increases the risk of developing symptomatic spinal stenosis. However, there is no consensus on the optimal management of TLK.
This retrospective cohort study evaluated children under four years old with achondroplasia, monitoring TLK every six months. Pathologic TLK was defined as a Cobb angle of 20 degrees or more between T10 and L2. Management involved either a wait-and-see policy, which prohibited unsupported sitting, or bracing. Surgery was reserved for severe progressive TLK or spinal stenosis cases. TLK was evaluated over time. A receiver operating characteristic curve determined the baseline threshold where wait-and-see management failed to resolve TLK below 20 degrees. Multiple linear regression compared bracing versus wait-and-see management for cases exceeding 40 degrees.
Sixty-two patients were included, with a median age of 10 months and a median follow-up of 31 months. TLK prevalence decreased from 85% at baseline to 42% at final follow-up. The mean Cobb angle decreased from 31 ± 11 degrees to 22 ± 16 degrees (p < 0.001). The threshold for ineffective wait-and-see management was identified as 33 degrees. Bracing resulted in significantly more TLK reduction than wait-and-see management for cases exceeding 40 degrees (15 degree difference, 95% CI 2-28, p = 0.023). Three patients required surgery.
TLK is highly prevalent in achondroplasia, necessitating careful monitoring. A wait-and-see policy with restrictions on unsupported sitting is recommended initially, but early bracing should be considered for more severe cases.
胸腰椎后凸（TLK）在软骨发育不全儿童中经常被报道。软骨发育不全中TLK与狭窄椎管的结合增加了出现症状性椎管狭窄的风险。然而，对于TLK的最佳治疗尚无共识。
这项回顾性队列研究评估了4岁以下的软骨发育不全儿童，每六个月监测一次TLK。病理性TLK定义为T10和L2之间的Cobb角为20度或更大。治疗方法包括观察等待策略（禁止无支撑坐姿）或支具治疗。手术仅用于严重进行性TLK或椎管狭窄病例。对TLK进行长期评估。通过绘制受试者工作特征曲线来确定观察等待治疗未能将TLK缓解至20度以下的基线阈值。对超过40度的病例，采用多元线性回归比较支具治疗与观察等待治疗的效果。
纳入62例患者，中位年龄为10个月，中位随访时间为31个月。TLK患病率从基线时的85%降至最终随访时的42%。平均Cobb角从31±11度降至22±16度（p<0.001）。确定观察等待治疗无效的阈值为33度。对于超过40度的病例，支具治疗导致的TLK改善明显大于观察等待治疗（相差l5度，95%CI 2-28，p=0.023）。3例患者需要手术治疗。
TLK在软骨发育不全中非常普遍，需要仔细监测。最初建议采用限制无支撑坐姿的观察等待策略，但对于更严重的病例应考虑早期使用支具治疗。
