Tag Archive | Duchenne muscular dystrophy

What’s New in Pediatric Orthopaedics: Level I and II Studies

Every month, JBJS publishes a Specialty Update—a review of the most pertinent and impactful studies published in the orthopaedic literature during the previous year in 13 subspecialties. Here is a summary of selected findings from Level I and II studies cited in the February 18, 2015 Specialty Update on pediatric orthopaedics:

Spine

–The landmark BrAIST study found that bracing helps prevent adolescent idiopathic scoliosis curves from progressing to a surgical range (≥50°), with a number needed to treat of 3. (See related OrthoBuzz article.)

–A randomized trial comparing the SpineCor brace to rigid bracing for correction of scoliosis found that the rate of curve progression was significantly higher in the SpineCor group.

Neuromuscular Conditions

–A study on the role of steroids in patients with Duchenne muscular dystrophy found that glucocorticoid therapy decreased the need for spinal surgery to treat scoliosis.

Trauma

–A randomized trial among patients 4 to 12 years of age with a distal radial or distal both-bone fracture found that the use of a double-sugar-tong splint for immediate post-reduction immobilization was at least as effective as the use of a plaster long arm cast.

–A randomized controlled trial of 61 patients from 5 to 12 years old who had a supracondylar humeral fracture found no functional or elbow-motion benefits associated with hospital-based physical therapy after short-term casting.

Foot and Ankle

–A randomized trial of 27 children less than 9 months of age who had resistant metatarsus adductus found that a group receiving orthotic treatment had greater improvement in footprint heel bisector measurements than those receiving serial casting. The orthotic program required more active parental participation but was about half the cost of casting.

–A randomized study of children under 3 months of age with idiopathic clubfoot who were treated with the Ponseti method found that the failure rates and treatment times were significantly higher in a below-the-knee casting group than in an above-the-knee casting group.

Editor’s Choice – April 7, 2014

How to prevent pulmonary function deterioration has been a focus in the management of boys with Duchenne muscular dystrophy (DMD) for many years. Since the 1980s it has been thought that an increasing scoliosis is associated with declining pulmonary function at a rate even greater than that from the effects of muscle weakness. As a result, it is common for surgery to be recommended for patients with DMD once a scoliosis of greater than 20 degrees is noted, a much lower threshold than is used for surgical treatment of idiopathic scoliosis. This practice assumes that surgical correction reduces the worsening of pulmonary function, but solid data to support that view has been absent.

The article “Functional Outcomes in Duchenne Muscular Dystrophy Scoliosis” in the March 5, 2014 JBJS confirms that surgical treatment of scoliosis in DMD does lead to better vital capacity, compared with no surgical treatment. However, before deciding that all DMD patients will need spine surgery to slow down pulmonary function worsening, surgeons should keep in mind the current efficacy of early treatment with corticosteroids to prevent scoliosis in this patient group. Not only does corticosteroid treatment prevent scoliosis development in the majority of kids, but the deterioration in pulmonary function is also slowed compared to those without this treatment.

With the information on pulmonary function provided in this article, we now have concrete data for use in discussions with parents on whether to select early treatment with corticosteroids to prevent scoliosis or to wait for surgical correction later. Surgery has risks associated with cardiac and pulmonary compromise inherent in DMD, and corticosteroids carry the risk of stunted growth and the development of cataracts in many patients. This article contributes useful hard data to enhance the process of shared decision making for the spinal care of children with DMD.