Management and Outcomes of Femur Fractures in Patients with Duchenne Muscular Dystrophy

Abstract

Background: 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. Methods: 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. Results: 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 (Group A) and six fractures in five patients underwent nonoperative management (Group B). 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. Conclusion: 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.