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 mo...

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Bibliographic Details
Published in:European spine journal 2006-04, Vol.15 (4), p.433-439
Main Authors: Martin, Anthony Gwynne, Foguet, Pedro R, Marks, David S, Thompson, A G, Child, A H
Format: Article
Language:English
Subjects:
Child, Preschool
Connective Tissue Diseases - diagnostic imaging
Connective Tissue Diseases - surgery
Female
Humans
Infant
Original
Orthopedic Fixation Devices
Orthopedic Procedures - instrumentation
Orthopedic Procedures - methods
Radiography
Scoliosis - diagnostic imaging
Scoliosis - etiology
Scoliosis - surgery
Syndrome
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Summary: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.
ISSN:0940-6719
1432-0932
DOI:10.1007/s00586-005-0980-9