Lower limb surgery in Duchenne muscular dystrophy.
Two hundred and thirteen of 428 patients with Duchenne muscular dystrophy (DMD) of a prospective and open study were operated on bilaterally with hip and knee release, aponeurectomy of the iliotibial band and Achilles tendon lengthening. In 87 patients the operation was carried out during early restrictions of the lower limb joint mobility at an average age of 6.56 years (4.02-8.26, SD 1.42). The follow-up was on average 5.4 years (0.25-9.01, SD 2.7). This group was compared to a control group (natural history) consisting of 100 non-operated DMD patients. A significant (P < 0.001) release of the contractures could be obtained. Loss of walking ability occurred in the control group at an average of 9.29 years (5.85-13.63, SD 1.98) and in the operated group at an average of 10.55 years (8.17-14.39, SD 1.76). This shows that early lower limb surgery leads to a prolongation of independent ambulation of 1.25 years on average. In contrast to the patients of the control group all treated patients between ages 6 and 8 years could walk independently. The positive influence of early lower limb surgery could also be shown by the development of Hammersmith motor ability score, CIDD (Council of Investigation of Duchenne Dystrophy) grading and Vignos scale. Nevertheless, in consideration of the well-known course of DMD not only the prolongation of ambulation but also the achieved prolongation of assisted standing ability with no or mild contractures are aims of lower limb surgery. Since no improvement of muscle strength could be observed after lower limb surgery, further studies have to investigate if additionally administered steroids can prolong ambulation after early lower limb surgery.
Forst
J
J
Orthopaedic Department, University Hospital RWTH Aachen, Germany. j.forst@t-online.de
Forst
R
R
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D007627:Keloid / Q000209:etiology; D007866:Leg / Q000601:surgery*; D009136:Muscular Dystrophies / Q000503:physiopathology / Q000601:surgery*; D011183:Postoperative Complications; D011446:Prospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D016138:Walking / Q000502:physiology