Clinical Results of Early Orthopaedic Management in Duchenne Muscular Dystrophy

Abstract

Although the primary defect of Duchenne muscular dystrophy has been found, there is no causal treatment to alter the natural course of this disease.

Based on the recommendations by Glorion and Rideau with early treatment of contractures of the hips and the lower limbs we performed a modified release of the spina muscles, resection of tensor fasciae latae muscle and a lengthening of the tendo calcaneus in 32 patients. The mean age of DMD patients at time of operation was 6.1 years. The mean follow-up was 3.4 years. All children underwent mobilisation the day after surgery. Complete correction of all contractures was immediately achieved after operation and kept in all but two cases up to the follow-up examination after 3.4 years. No loss of ambulation was observed.

Our results demonstrate that early selective surgery in DMD patients just at or better before the onset of contractures without performing an additional aponeurectomy of the iliotibial band and percutaneous tenotomy of the hamstrings according to the original Glorion-Rideau technique safely prevents severe contractures and thereby delays the progression of scoliosis.

Key words

Duchenne muscular dystrophy - Surgery