The authors sought to determine the overall incidence of cervicomedullary decompression (CMD) in patients with achondroplasia and the characteristics associated with those surgeries across multiple institutions with experience caring for individuals with skeletal dysplasias.

Data from CLARITY (Achondroplasia Natural History Study) for 1374 patients with achondroplasia from four skeletal dysplasia centers (A. I. duPont Hospital for Children, Johns Hopkins University, University of Texas Health, and University of Wisconsin School of Medicine and Public Health) followed from 1957 to 2017 were recorded in a Research Electronic Data Capture (REDCap) database. Data collected and analyzed included surgeries, indications, complications, ages at time of procedures, screening procedures, and medical diagnoses.

There were 314 CMD procedures in 281 patients (20.5% of the entire cohort). The median age of first CMD was 1.3 years in males and 1.1 years in females. Over time, there was a decrease in the median age of patients at first CMD. All patients born before 1980 who underwent CMD had the procedure after 5 years of age, whereas 98% of patients born after 2010 underwent CMD before 5 years of age. In addition, a greater proportion of patients born in more recent decades had documented neuroimaging and polysomnography (PSG) prior to CMD. Ventriculoperitoneal shunts (VPSs) were placed more frequently in patients undergoing CMD (23%) than in the entire cohort (8%). Patients who required either CMD or VPS were 7 times more likely to require both surgeries than patients who required neither surgery (OR 7.0, 95% CI 4.66–10.53; p < 0.0001). Overall, 10.3% of patients who underwent CMD required a subsequent CMD.

The prevalence of CMD in this large achondroplasia cohort was 20%, with more recently treated patients undergoing first CMD at younger ages than earlier patients. The use of neuroimaging and PSG screening modalities increased over time, suggesting that increased and better surveillance contributed to earlier identification and intervention in patients with cervicomedullary stenosis and its complications.

作者试图确定软骨发育不全患者的颈髓减压 (CMD) 的总体发生率，以及与这些手术相关的特征，这些特征来自多个机构，这些机构具有护理骨骼发育不良患者的经验。

来自 CLARITY（软骨发育不全自然史研究）的数据来自四个骨骼发育不良中心（AI 杜邦儿童医院、约翰霍普金斯大学、德克萨斯大学健康和威斯康星大学医学和公共卫生学院）的 1374 名软骨发育不全患者，从 1957 年到2017 年记录在研究电子数据捕获 (REDCap) 数据库中。收集和分析的数据包括手术、适应症、并发症、手术时的年龄、筛查程序和医疗诊断。

281 名患者（占整个队列的 20.5%）进行了 314 次 CMD 手术。首次 CMD 的中位年龄男性为 1.3 岁，女性为 1.1 岁。随着时间的推移，首次 CMD 患者的中位年龄有所下降。1980 年之前出生的所有接受 CMD 的患者在 5 岁之后接受了手术，而 2010 年之后出生的患者中有 98% 在 5 岁之前接受了 CMD。此外，最近几十年出生的更大比例的患者在 CMD 之前记录了神经影像学和多导睡眠图 (PSG)。接受 CMD 的患者 (23%) 比整个队列 (8%) 更频繁地放置脑室腹腔分流术 (VPS)。需要 CMD 或 VPS 的患者需要两种手术的可能性是既不需要手术的患者的 7 倍（OR 7.0，95% CI 4.66–10.53；p < 0.0001）。总的来说，10。

在这个大型软骨发育不全队列中，CMD 的患病率为 20%，与早期患者相比，最近接受治疗的患者在更年轻的时候接受了首次 CMD。神经影像学和 PSG 筛查方式的使用随着时间的推移而增加，这表明增加和更好的监测有助于早期识别和干预颈髓狭窄及其并发症的患者。