Martin Anthony Gwynne, Foguet Pedro R, Marks David S, Thompson A G, Child A H
Spinal Surgery, Royal Orthopaedic Hospital, Birmingham, UK.
Eur Spine J. 2006 Apr;15(4):433-9. doi: 10.1007/s00586-005-0980-9. Epub 2005 Sep 20.
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.
比尔斯综合征(先天性挛缩性蜘蛛指)是一种结缔组织的遗传性疾病,在表型上与马方综合征相关。其特征为肢体细长、蜘蛛指、多关节挛缩、皱缩耳、肌肉发育不全和脊柱侧弯。脊柱侧弯是这种罕见病症最重要的临床特征,出现在婴幼儿和青少年年龄组,且有迅速进展的趋势。支具治疗往往无法控制脊柱侧弯,手术是推荐的治疗方法。我们介绍了两例采用小儿伊索拉器械和非融合技术治疗的经验。