Importance of lower limb surgery in Duchenne muscular dystrophy. 1995

R Forst, and J Forst
Orthopädische Klinik RWTH Aachen, Germany.

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 lengthening. In 57 patients (group I) 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 II) 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 assess the interindividual course of both groups, we defined "joint and motor quotients", which allowed a complex assessment of joint function and motoric capacity. 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 II. 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.(ABSTRACT TRUNCATED AT 250 WORDS)

UI MeSH Term Description Entries
D007866 Leg The inferior part of the lower extremity between the KNEE and the ANKLE. Legs
D009136 Muscular Dystrophies A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS. Muscular Dystrophy,Myodystrophica,Myodystrophy,Dystrophies, Muscular,Dystrophy, Muscular,Myodystrophicas,Myodystrophies
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003286 Contracture Prolonged shortening of the muscle or other soft tissue around a joint, preventing movement of the joint. Contractures
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012600 Scoliosis An appreciable lateral deviation in the normally straight vertical line of the spine. (Dorland, 27th ed) Scolioses

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