IMPORTANCE OF LOWER-LIMB SURGERY IN DUCHENNE MUSCULAR-DYSTROPHY

A total of 123 patients with Duchenne muscular dystrophy (DMD) was sur gically treated during two different periods of their course by hip an d knee release, aponeurectomy of the iliotibial band and z-shaped Achi lles' tendon lenghtening. In 57 patients (group I) this was carried ou t prophylactically as retractions of the lower limb joints were just b eginning at the age of 6.4 +/- 1.43 years and in 66 patients (group II ) as mild contractures of the joints at the end of walking ability wer e already manifest with an average age of 9.27 +/- 1.86 years. The ave rage follow-up was 3.7 +/- 1.2 years in both groups. To be able to ass es the interindividual course of both groups, we defined ''joint and m otor quotients'', which allowed a complex assessment of joint function and motoric capacity. In addition, both groups were compared with a c ontrol group (natural history) consisting of 100 non-operated DMD pati ents. In both groups a significant release of the contractures could b e obtained primarily. Patients in group I showed a much better long-te rm effect than those in group II. The motor quotient in group I was si gnificantly 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 defo rmities. In particular, maintenance of standing ability for patients c onfined to a wheelchair leads to a proven, significantly delayed devel opment of the fatal scoliosis and thus to an essential improvement of the patient's quality of life after having lost the ability to walk. I n summary, our results showed that lower limb surgery in DMD should be recommended as routine in principle especially at an early stage of t he disease.