Infantile scoliosis in Beals syndrome: the use of a non-fusion technique for surgical correction.
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.
Martin
Anthony Gwynne
AG
Spinal Surgery, Royal Orthopaedic Hospital, Birmingham, UK. antmartinincardiff@hotmail.com
Foguet
Pedro R
PR
Marks
David S
DS
Thompson
A G
AG
Child
A H
AH
D002675:Child, Preschool; D003240:Connective Tissue Diseases / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D009984:Orthopedic Fixation Devices; D019637:Orthopedic Procedures / Q000295:instrumentation / Q000379:methods*; D011859:Radiography; D012600:Scoliosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery*; D013577:Syndrome