European Spine Journal

, Volume 15, Issue 4, pp 433–439

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

  • Anthony Gwynne Martin
  • Pedro R. Foguet
  • David S. Marks
  • A. G. Thompson
  • A. H. Child
Original Article

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.

Keywords

Beals syndrome Congenital contractural arachnodactyly Fibrillin Progressive infantile scoliosis Posterior spinal instrumentation without fusion 

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Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Anthony Gwynne Martin
    • 1
  • Pedro R. Foguet
    • 1
  • David S. Marks
    • 1
  • A. G. Thompson
    • 1
  • A. H. Child
    • 2
  1. 1.Royal Orthopaedic HospitalSpinal SurgeryBirminghamUK
  2. 2.St. Georges HospitalGeneticsLondonUK

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