PDF(Pubmed)
Abstract：
OBJECTIVE: Arthrogryposis multiplex congenita (AMC) consists of more than 400 conditions involving severe joint contractures of at least two or more body regions. Management of clubfoot in patients with AMC is notoriously challenging, with a higher likelihood of recurrence than idiopathic clubfoot, which can be treated using the Ponseti technique to avoid or delay more invasive procedures. The purpose of this study is to determine the utility of multiple serial casting as a treatment of clubfoot in AMC using Pirani scores as an objective measure of deformity.
METHODS: Pirani scores were retrospectively collected from 17 AMC patients with a total of 30 clubfeet and two years follow-up from initiation of treatment. Patients with a minimum of three casting series were included. Pre-treatment and post-treatment deformity scores were examined across casting series using analysis of variance (ANOVA) statistical analysis.
RESULTS: The first series pre-treatment Pirani score improved from 4.80±1.54 to 1.68±1.48 (p<0.001). The second series improved from 4.23±1.03 to 2.72±0.916 (p<0.001). The third series had the smallest improvement from 3.87±1.07 to 2.82±1.02 (p<0.001). Change in Pirani scores showed a significant decrease from the first series to the second (p=0.001) and third (p<0.001). In addition, the number of casting days was found to significantly affect the change in scores during the third series (p=0.038).
CONCLUSIONS: The Ponseti technique can improve clubfoot in AMC as measured by the Pirani score. Data shows that early intervention yields better results, with a diminished yet effective ability to elicit change over time.
METHODS: Pirani scores were retrospectively collected from 17 AMC patients with a total of 30 clubfeet and two years follow-up from initiation of treatment. Patients with a minimum of three casting series were included. Pre-treatment and post-treatment deformity scores were examined across casting series using analysis of variance (ANOVA) statistical analysis.
RESULTS: The first series pre-treatment Pirani score improved from 4.80±1.54 to 1.68±1.48 (p<0.001). The second series improved from 4.23±1.03 to 2.72±0.916 (p<0.001). The third series had the smallest improvement from 3.87±1.07 to 2.82±1.02 (p<0.001). Change in Pirani scores showed a significant decrease from the first series to the second (p=0.001) and third (p<0.001). In addition, the number of casting days was found to significantly affect the change in scores during the third series (p=0.038).
CONCLUSIONS: The Ponseti technique can improve clubfoot in AMC as measured by the Pirani score. Data shows that early intervention yields better results, with a diminished yet effective ability to elicit change over time.
摘要：
目的：多发性先天性关节病（AMC）由400多种疾病组成，涉及至少两个或多个身体区域的严重关节挛缩。众所周知，AMC患者的马蹄内翻足治疗具有挑战性，复发的可能性比特发性马蹄内翻足高,可以使用Ponseti技术进行治疗，以避免或延迟更多的侵入性程序。这项研究的目的是使用Pirani评分作为畸形的客观衡量标准，确定多次连续铸造作为AMC中马蹄内翻足治疗的实用性。
方法：回顾性收集了17例AMC患者的皮拉尼评分，这些患者共30只马蹄，并在治疗开始后进行了两年的随访。包括至少三个铸造系列的患者。使用方差分析（ANOVA）统计分析检查整个铸件系列的治疗前和治疗后畸形评分。
结果：第一系列治疗前的皮拉尼评分从4.80±1.54提高到1.68±1.48（p<0.001）。第二个系列从4.23±1.03提高到2.72±0.916(p<0.001)。第三个系列的改善最小，从3.87±1.07降至2.82±1.02（p<0.001）。皮拉尼评分的变化显示，从第一个系列到第二个系列（p=0.001）和第三个系列（p<0.001）显着降低。此外,在第三系列中，铸造天数显着影响分数的变化（p=0.038）。
结论：通过Pirani评分测量，Ponseti技术可以改善AMC中的马蹄内翻足。数据显示，早期干预效果更好，随着时间的推移，引起变化的能力减弱但有效。
方法：回顾性收集了17例AMC患者的皮拉尼评分，这些患者共30只马蹄，并在治疗开始后进行了两年的随访。包括至少三个铸造系列的患者。使用方差分析（ANOVA）统计分析检查整个铸件系列的治疗前和治疗后畸形评分。
结果：第一系列治疗前的皮拉尼评分从4.80±1.54提高到1.68±1.48（p<0.001）。第二个系列从4.23±1.03提高到2.72±0.916(p<0.001)。第三个系列的改善最小，从3.87±1.07降至2.82±1.02（p<0.001）。皮拉尼评分的变化显示，从第一个系列到第二个系列（p=0.001）和第三个系列（p<0.001）显着降低。此外,在第三系列中，铸造天数显着影响分数的变化（p=0.038）。
结论：通过Pirani评分测量，Ponseti技术可以改善AMC中的马蹄内翻足。数据显示，早期干预效果更好，随着时间的推移，引起变化的能力减弱但有效。
