Affiliations: [a] Department of Physical Therapy, Children’s Hospital of Philadelphia, Philadelphia PA | [b] Center for Rehabilitation, Children’s Hospital of Philadelphia, Philadelphia PA | [c] Division of Neurology, Children’s Hospital of Philadelphia, Philadelphia PA | [d] Department of Orthopedics, Children’s Hospital of Philadelphia, Philadelphia PA | [e] Perelman School of Medicine at The University of Pennsylvania, Philadelphia PA
Correspondence to: Allan M. Glanzman, Children’s Hospital of Philadelphia, 3400 Civic Center Blvd.,
Philadlephia, PA 19104. Tel.: +1 215 590 7730; E-mail: [email protected].
Abstract: Background:Boys with dystrophinopathies (DMD) are at increased risk of low bone mineral density and fracture. Femoral fracture is the most common extremity fracture and is accompanied by significant risk of functional loss. Care considerations for DMD have stressed that aggressive management may be needed to maintain ambulation and that surgical fixation allows early mobilization. Objectives:Describe 5 cases of femoral fracture in ambulatory boys with DMD and the course of care undertaken to optimize function. Patients:Five boys with DMD median age 15y (12–16) who were independently ambulatory. Median 10m walk speed prior to their first fracture was 8 sec (7–17.37) and 4 of 5 were less than the 9 seconds predictive of 2 year ambulation retention. Three of the cases had a single incident causing fracture; the remaining cases had 2 and 3 incidents respectively representing a total of 8 fractures 6 of which were surgically stabilized. Results:Following the first fracture, all 5 subjects regained some form of ambulation. Three patients regained independent ambulation and 2 with hand held support or contact guard. Two subjects went on to have additional falls with associated fracture. No patient regained the ability to rise from the floor and only one of the 5 regained the ability to climb steps and all demonstrated a decline in walking speed. Conclusion:Prompt orthopedic intervention, early mobility, and intensive rehabilitation even in the end stage ambulatory patient, were factors in helping preserve function in these patients with dystrophinopathies.