THERAPEUTIC ASPECTS OF SCOLIOSIS IN MUSCL E DISORDERS

Introduction: Patients suffering from the most frequent muscle disorde rs Duchenne muscular dystrophy (DMD) and spinal muscular atrophies (SM A), who ceased walking respectively are confined from the outset to th e wheel-chair, are developing commonly a progressive scoliosis (collap sing spine) due to an increasing muscle weakness. Basing on the pelvic obliquity these scolioses are leading first of all to problems in sit ting as well as difficulties in trunc and head control. Along with the increasing weakness of respiratory muscles these phenomena entail a r estrictive respiratory insufficiency. Conservative treatment: An effec tive conservative treatment is not available for these scolioses. The use of a corset, however, can only be taken into consideration as a co mpromise, either for very young patients or those who refused an opera tion respectively who have reached an inoperable stage. The exclusive use of so-called ''anatomic sitting supports'' in the wheel-chair in o rder to treat or prevent a progressive scoliosis in DMD or SMA is abso lutely to be rejected. They should only be applied for very young pati ents with SMA type II as a transitional solution until a corset or bet ter an surgical stabilisation of the spine will be effected, or as a p alliative measure in late stages. Surgical treatment: Only the early a s possible performed surgical stabilisation of the spine using adequat e instrumentation (Luque, CD or modifications), enabling an early mobi lization without corset or cast, is the most effective treatment of th ese scoliosis. Patients with DMD or SMA type III should be stabilized after loss of walking ability and definitive confinement to wheel-chai r, if the curve is more than 20 degrees-30 degrees Cobb and progressiv e and forced vital capacity (FVC) is >35%, The instrumentation should be applied between D3 or D4 and sacrum. The bony fusion mass should in clude the lumbar and lumbosacral region. The unfusioned instrumentatio n with the telescope-rod after Naumann is a good solution for patients with SMA type II and progressive curves already in the early childhoo d from ca. 5 years of age. First of all surgical spinal stabilisation improves the sitting comfort. Over and above this the improved cosmeti c appearance should not be underestimated for the psychological condit ion of these patients. Additionally it is proved, that surgical stabil isation of the spine prolongs the life expectancy of patients with DMD . Furthermore stabilization of lung function can be achieved for both DMD and SMA patients in comparison to the natural history of these dis eases. Altogether a decisive improvement of quality of life can be rea ched for all these patients.