Importance of lower limb surgery in Duchenne muscular dystrophy

Abstract

A total of 123 patients with Duchenne muscular dystrophy (DMD) was surgically treated during two different periods of their course by hip and knee release, aponeurectomy of the iliotibial band and z-shaped Achilles' tendon lenghtening. In 57 patients (group 1) this was carried out prophylactically as retractions of the lower limb joints were just beginning at the age of 6.4 ± 1.43 years and in 66 patients (group 11) as mild contractures of the joints at the end of walking ability were already manifest with an average age of 9.27 ± 1.86 years. The average follow-up was 3.7 ± 1.2 years in both groups. To be able to asses the interindividual course of both groups, we defined “joint and motor quotients”, which allowed a complex assessment of joint function and motoric capactiy. In addition, both groups were compared with a control group (natural history) consisting of 100 non-operated DMD patients. In both groups a significant release of the contractures could be obtained primarily. Patients in group I showed a much better long-term effect than those in group 11. The motor quotient in group I was significantly better over the whole follow-up period (P < 0.001) than in group II or the control group. The prolongation of walking ability by about 2 years compared with the natural history is in our opinion not the central goal of this surgical treatment concept of lower limbs in DMD, but rather the additionally achieved prolongation of an assisted standing ability with the lower limbs free from contractures and deformities. In particular, maintenance of standing ability for patients confined to a wheelchair leads to a proven, significantly delayed development of the fatal scoliosis and thus to an essential improvement of the patient's quality of life after having lost the ability to walk. In summary, our results showed that lower limb surgery in DMD should be recommended as routine in principle especially at an early stage of the disease.