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.