| Scoliosis in patients with Friedreich's ataxia. | |
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MedLine Citation:
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PMID: 22529092 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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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. |
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Authors:
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A I Tsirikos; G Smith |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: The Journal of bone and joint surgery. British volume Volume: 94 ISSN: 0301-620X ISO Abbreviation: J Bone Joint Surg Br Publication Date: 2012 May |
Date Detail:
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Created Date: 2012-04-24 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0375355 Medline TA: J Bone Joint Surg Br Country: England |
Other Details:
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Languages: eng Pagination: 684-9 Citation Subset: AIM; IM |
Affiliation:
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Scottish National Spine Deformity Centre, Royal Hospital for Sick Children, Sciennes Road, Edinburgh EH9 1LF, UK. |
Export Citation:
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Descriptor/Qualifier:
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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