Orthopedic and Surgical Management of the Patient With Duchenne Muscular Dystrophy

Abstract

Duchenne muscular dystrophy (DMD) has common and predictable musculoskeletal manifestations. In addition to progressive muscle weakness and loss of function, patients may develop joint contractures, scoliosis, and fractures due to osteoporosis. The causes of these musculoskeletal complications are multifactorial and are related to primary effects on the The guidelines or recommendations in this article are not American Academy of Pediatrics policy and publication herein does not imply endorsement. Orthopedic care is an important aspect of the overall management of patients with Duchenne muscular dystrophy (DMD). In addition to progressive muscle weakness and loss of function, patients may develop joint contractures, scoliosis, and osteoporosis, causing fractures; all of these necessitate intervention by a multidisciplinary team including an orthopedic surgeon as well as rehabilitation specialists such as physio-and occupational therapists. The causes of these musculoskeletal complications are multifactorial and are related to primary effects on the muscles from the disease itself, secondary effects from weak muscles, and the related side effects of treatments, such as glucocorticoid use that affect bone strength. The musculoskeletal manifestations of DMD change over time as the disease progresses, and therefore, musculoskeletal management needs change throughout the life span of an individual with DMD. In this review, we target pediatricians, neurologists, orthopedic surgeons, rehabilitation physicians, anesthesiologists, and other individuals involved in the management of patients with DMD by providing specific recommendations to guide clinical practice related to orthopedic issues and surgical management in this setting.

Respiratory and upper limb function as outcome measures in ambulant and non-ambulant subjects with Duchenne muscular dystrophy: A prospective multicentre study

“…Scoliosis can develop due to weakness of trunk muscles and often requires surgical correction to maintain respiratory capacity. [6] Absence of dystrophin in the heart leads to cardiomyopathy, and respiratory muscle decline ultimately results in dependence on assisted ventilation. [7] Death is usually caused by cardiac and/or respiratory failure and related complications.…”

Flexible intramedullary nailing for supracondylar femoral fractures in children with Duchenne muscular dystrophy

“…The main issue for operative treatment in DMD is the considerable anesthetic risk due to the impairment of pulmonary and heart functions typical of the advanced stage of the disease [ 15 , 16 ]. In addition, prolonged glucocorticoid therapy reduces the stress response during surgery [ 17 , 18 ].…”

Guidance for the care of neuromuscular patients during the COVID-19 pandemic outbreak from the French Rare Health Care for Neuromuscular Diseases Network

“…Maintaining joint flexibility, muscle strength, and endurance is recommended in many neuromuscular diseases through regular in-house or office care by health professionals [4][5][6][7][8].…”