Infantile scoliosis in Beals syndrome: the use of a non-fusion technique for surgical correction

被引:3
|
作者
Martin, AG
Foguet, PR
Marks, DS
Thompson, AG
Child, AH
机构
[1] Royal Orthopaed Hosp, Birmingham B31 2AP, W Midlands, England
[2] St George Hosp, London, England
关键词
Beals syndrome; congenital contractural arachnodactyly; fibrillin; progressive infantile scoliosis; posterior spinal instrumentation without fusion;
D O I
10.1007/s00586-005-0980-9
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Beals syndrome (congenital contractural arachnodactyl) is a genetic disorder of the connective tissue phenotypically related to Marfan syndrome. It is characterised by dolichostenomelia, arachnodactyly, multiple joint contractures, crumpled ears, hypoplastic muscles and scoliosis. The latter, the most important clinical feature of this rare condition, presents in the infantile and juvenile age group and has a tendency to rapid progression. Bracing often fails to control the scoliosis and surgery is the recommended treatment. We present our experience of two cases managed with the paediatric Isola instrumentation and a non-fusion technique.
引用
收藏
页码:433 / 439
页数:7
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