Scoliosis in patients with Friedreich’s ataxia

A. I. Tsirikos, G. Smith

Abstract

We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities.

Footnotes

  • The authors would like to thank Mr M. J. McMaster, MD, FRCS, whose data contributed to this study.

    No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article

  • Supplementary material. A table detailing the demographic data and results of each of the 31 patients is available with the electronic version of this article on our website www.jbjs.boneandjoint.org.uk

  • Received September 29, 2011.
  • Accepted January 25, 2012.
View Full Text

Log in through your institution