title: Infantile scoliosis in Beals syndrome: the use of a non-fusion technique for surgical correction.
abstract: 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.
authors.affiliation: Spinal Surgery, Royal Orthopaedic Hospital, Birmingham, UK. antmartinincardiff@hotmail.com
authors.forename: Anthony Gwynne
authors.initials: AG
authors.lastname: Martin
authors.forename: Pedro R
authors.initials: PR
authors.lastname: Foguet
authors.forename: David S
authors.initials: DS
authors.lastname: Marks
authors.forename: A G
authors.initials: AG
authors.lastname: Thompson
authors.forename: A H
authors.initials: AH
authors.lastname: Child
mesh_terms: 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