Fractures in proximal spinal muscular atrophy.
Fractures are a common problem for patients with spinal muscular atrophy (SMA). A total of 131 patients with proximal SMA with an average age of 13.2 +/- 9.2 years (0.7-65.6) were evaluated retrospectively. In 60 patients 94 different fractures were observed. The group consisted of 11 patients with type Ib, 81 with type II, 33 with type IIIa, 4 with IIIb and 2 with type IV. 38 of 81 SMA II patients and 17 of 33 SMA IIIa patients had suffered fractures at an average age of 8.3 +/- 5.3 years (0.0-25.1) (SMA II) and 9.3 +/- 6.0 years (0.0-22.1) (SMA IIIa). The most frequent fractures were of the femur (50), usually distal, of the lower leg and ankle (15), and upper arm (9). The distribution of fractures was different in SMA II and SMA IIIa. Most of the fractures could be treated conservatively. Only two femoral shaft fractures, one upper arm and a lower arm fracture were treated surgically by osteosynthesis. Competent fracture treatment is an important part of the orthopaedic care of SMA patients.