Traitement chirurgical des scolioses de la dystrophie musculaire de Duchenne

Abstract

Purpose of the study The purpose of this retrospective study is to demonstrate the advantages of early surgical operation for patients suffering from Duchenne muscular dystrophy scoliosis. Patients and methods Since 1992, 37 patients suffering from Duchenne muscular dystrophy were operated on for scoliosis. Mean age was 12 years. Vital capacity was 62 ± 17% and left ventricular ejection fraction 55 ± 7%. Insertion of flexible vertebral instrumentation included a pedicular screwing system in the lumbo-sacral area and transversal attachments with steel threads at the thoracic level. A sub-laminar fastening was placed at L1. Bone bank arthrodesis was performed only at lumbo-sacral level, in order to maintain flexibility in the thoracic part of the assembly and to enable growth. Results Assisted ventilation was necessary in three children during 1.5 month. Superficial sepsis was treated locally with an antibiotherapy without the removal of material in four patients. There was one stem rupture two years after operation, caused by a road traffic accident. No further procedure was necessary for technical reasons. There was no death during the longest follow-up period among the first 24 patients (mean follow-up: 57 months). In the frontal plane, the preoperative Cobb angle, which was 19°, was brought to 5.2° at the postoperative stage, and 9.5% at the latest measurement, i.e., a loss of angular correction of 4.3°. In the sagittal plane, there were physiological curvatures. Pelvic balancing was correct and results have held over time. Vital capacity was reduced by 3.6% per year. Conclusion These results encourage early operation on these patients in order to avoid anaesthetic, peri- and postoperative complications. Likewise, giving support to minor curves reduces mechanical constraints during the first postoperative years. The absence of thoracic arthrodesis enables growth of about 5 cm when patients are operated on at about the age of 12 years. Stabilization of the myopath's spine enables the child to remain in an upright sitting position. The assembly's thoracic suppleness enables an increase in the range of movement in the upper limbs. It seems appropriate to operate on such patients when they cease walking, around the age of 12 years. Cardiorespiratory function and life expectancy are not improved, but most patients and families are very satisfied by the comfort brought about by the surgical operation.