Treatment of scoliosis in intermediate spinal muscular atrophy (SMA type II) in childhood.

Summary. Progressive scoliosis with increasing pelvic obliquity in early childhood of patients with SMA type II is a common feature in this disease. Spinal surgery in muscle disorders should be carried out as soon as a progressive curve of more then 20 Celsius Cobb and a preserved FVC of 20-30% is proved. In later stages or severe forms of SMA II spinal stabilization becomes often impossible due to the respiratory insufficiency, the poor general condition and the severity of the scoliosis with marked pelvic obliquity. A special telescope rod was developed in order to enable a lengthening of this instrumentation during growth for children treated in early childhood. In 15 of 20 patients with SMA II in early childhood not satisfactory results after telescope rod implantation were observed. In spite of the telescope technique crankshaft phenomenon appeared and curve progression were observed. So then we stopped telescope rod implantation. This instrumentation could be in principle a good therapeutical tool for this indication, but its technical manufacturing has firstly to be improved decisively. For SMA II patients younger than 10 years with progressive scoliosis our therapeutic recommendation is nowadays a corset until the age of 10-12 years followed by definitive surgical correction using other multisegmental instrumentation like the Isola(R) system.