Fractures in proximal spinal muscular atrophy

被引:19
作者
Fujak, Albert [1 ]
Kopschina, Carsten [2 ]
Forst, Raimund [1 ]
Gras, Florian [3 ]
Mueller, Lutz Arne [1 ]
Forst, Juergen [1 ]
机构
[1] Univ Erlangen Nurnberg, Dept Orthopaed Surg, D-91054 Erlangen, Germany
[2] Klinikum Nurnberg Sud, Dept Trauma & Orthopaed Surg, D-90471 Nurnberg, Germany
[3] Univ Jena, Dept Trauma Hand & Reconstruct Surg, D-07740 Jena, Germany
关键词
Spinal muscular atrophy; Fractures; Distal fractures of the femur; Osteopenia; Inactivity; Contractures; CONGENITAL BONE-FRACTURES; VARIANT; LETHAL; SMA;
D O I
10.1007/s00402-010-1096-1
中图分类号
R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学(修复外科学)];
学科分类号
摘要
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 +/- A 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 +/- A 5.3 years (0.0-25.1) (SMA II) and 9.3 +/- A 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.
引用
收藏
页码:775 / 780
页数:6
相关论文
共 19 条
[1]  
Ballestrazzi A., 1989, CURRENT CONCEPTS CHI, P215
[2]   INFANTILE SPINAL MUSCULAR-ATROPHY (SMA) AND MULTIPLE CONGENITAL BONE-FRACTURES IN SIBS - A LETHAL NEW SYNDROME [J].
BOROCHOWITZ, Z ;
GLICK, B ;
BLAZER, S .
JOURNAL OF MEDICAL GENETICS, 1991, 28 (05) :345-348
[3]   Infantile spinal muscular atrophy variant with congenital fractures in a female neonate: evidence for autosomal recessive inheritance [J].
Courtens, W ;
Johansson, AB ;
Dachy, B ;
Avni, F ;
Telerman-Toppet, N ;
Scheffer, H .
JOURNAL OF MEDICAL GENETICS, 2002, 39 (01) :74-77
[4]   Severe spinal muscular atrophy variant associated with congenital bone fractures [J].
Felderhoff-Mueser, U ;
Grohmann, K ;
Harder, A ;
Stadelmann, C ;
Zerres, K ;
Bührer, C ;
Obladen, M .
JOURNAL OF CHILD NEUROLOGY, 2002, 17 (09) :718-721
[5]  
Forst R, 2003, ORTHOPADIE ORTHOPADI, P243
[6]   Proximal spinal muscular atrophy (SMA) [J].
Fujak, A. ;
Wollinsky, K. H. ;
Forst, R. .
ZEITSCHRIFT FUR ORTHOPADIE UND UNFALLCHIRURGIE, 2007, 145 (02) :233-248
[7]  
GRANATA C, 1991, Chirurgia degli Organi di Movimento, V76, P39
[8]  
Hsu J D, 1982, Prog Clin Biol Res, V101, P553
[9]  
HSU JD, 1979, JOHNS HOPKINS MED J, V145, P89
[10]  
Kelly TE, 1999, AM J MED GENET, V87, P65, DOI 10.1002/(SICI)1096-8628(19991105)87:1<65::AID-AJMG13>3.0.CO