Gajewski Christopher R, Chen Kevin Y, Chang Eric, Levine Doug, Valdes Jennifer Wallace, Thompson Rachel M
UCLA Department of Orthopedic Surgery, Los Angeles, CA.
David Geffen School of Medicine at UCLA, Los Angeles, CA.
J Pediatr Soc North Am. 2024 Feb 12;5(3):664. doi: 10.55275/JPOSNA-2023-664. eCollection 2023 Aug.
Duchenne muscular dystrophy (DMD) is a severe, progressive X-linked recessive neuromuscular disorder characterized by muscle weakness and atrophy. Additionally, patients with DMD have significant reductions in bone mineral density compared to age-matched controls, which is exacerbated by concomitant steroid use. These findings dramatically increase fracture risk, which may irreparably decrease functional status. The aim of this case series is to examine outcomes of operative versus nonoperative management of femur fractures in this patient population. An IRB-approved retrospective chart review was completed for patients with DMD treated at a single institution for a femur fracture between 2013-2022. Patients were excluded for incomplete documentation, treatment initiation at an outside hospital, or diagnosis of a different muscular dystrophy. Demographic variables, treatment information, functional status, and adverse events were collected for each patient. Descriptive statistics were used to summarize demographic and outcome variables. A total of 10 patients with 11 femur fractures were included for analysis. All patients were male with an average age of 12.7 years and clinical follow-up of 286 days. Five fractures in five patients underwent operative fixation (), and six fractures in five patients underwent nonoperative management (). In Group A, three patients were short-distance ambulators prior to injury, and all patients regained a similar functional status postoperatively. All three patients were treated with a locked intramedullary nail. One patient in Group B was a short-distance ambulator prior to injury; the remainder were nonambulatory. All patients in Group B were primary wheelchair users at final follow-up. There were no adverse events as a result of treatment in either group. Nonoperative management with cast immobilization remains an acceptable option for nonambulatory patients and those with minimally displaced fractures not amenable to surgical intervention. Surgical intervention is recommended for higher-functioning patients with the goal of restoring ambulatory status. Regardless of treatment modality, patients should receive aggressive physical therapy directed at early weight-bearing, range of motion, and mobilization to preserve strength, muscle mass, and mobility. Level IV case series.
•Management of femur fractures in Duchenne muscular dystrophy should account for fracture morphology as well as patient functional status.•Surgical management is safe and effective in promoting fracture union and preserving functional status.•Nonoperative management is preferred in nonambulatory patients and for minimally displaced fractures.
杜氏肌营养不良症（DMD）是一种严重的、进行性的X连锁隐性神经肌肉疾病，其特征为肌肉无力和萎缩。此外，与年龄匹配的对照组相比，DMD患者的骨密度显著降低，同时使用类固醇会使这种情况恶化。这些发现显著增加了骨折风险，这可能会不可挽回地降低功能状态。本病例系列的目的是研究该患者群体中股骨骨折手术治疗与非手术治疗的结果。对2013年至2022年期间在单一机构接受股骨骨折治疗的DMD患者进行了一项经机构审查委员会批准的回顾性病历审查。排除记录不完整、在外部医院开始治疗或诊断为其他肌肉营养不良症的患者。收集了每位患者的人口统计学变量、治疗信息、功能状态和不良事件。使用描述性统计来总结人口统计学和结果变量。共有10例患者发生11处股骨骨折纳入分析。所有患者均为男性，平均年龄12.7岁，临床随访286天。5例患者的5处骨折接受了手术固定（）, 5例患者的6处骨折接受了非手术治疗（）. A组中，3例患者在受伤前为短距离步行者，所有患者术后恢复了相似的功能状态。所有3例患者均接受了带锁髓内钉治疗。B组中有1例患者在受伤前为短距离步行者；其余患者不能行走。B组所有患者在最后随访时均为主要轮椅使用者。两组治疗均未出现不良事件。对于不能行走的患者以及骨折移位极小、不适合手术干预的患者，采用石膏固定的非手术治疗仍然是一种可接受的选择。对于功能较高的患者，建议进行手术干预，目标是恢复步行状态。无论采用何种治疗方式，患者都应接受积极的物理治疗，包括早期负重、活动范围和活动能力训练，以保持力量、肌肉质量和活动能力。IV级病例系列。
•杜氏肌营养不良症患者股骨骨折的治疗应考虑骨折形态以及患者的功能状态。•手术治疗在促进骨折愈合和保持功能状态方面是安全有效的。•非手术治疗适用于不能行走的患者和骨折移位极小的患者。
