Comparison of Curve Shape Between Children With Cerebral Palsy, Friedreich's Ataxia, and Adolescent Idiopathic Scoliosis

Aronsson, David D.; Stokes, Ian A. F.; Ronchetti, Peter J.

doi:10.1111/j.1469-8749.1994.tb11867.x

Cited by 15 publications

(1 citation statement)

References 14 publications

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“…The pattern of deformity is suggested to resemble adolescent idiopathic scoliosis (AIS). 22 In our series, we recorded almost equal distribution of curves between single thoracic, thoracolumbar and double thoracic/lumbar with only two patients having long C-shaped collapsing scoliosis associated with pelvic obliquity, which is consistent with previous reports showing that 14% to 25% of patients with FA develop Cshaped thoracolumbar curves. [4][5][6][7]12 Hyperkyphosis has also been described with a frequency of 24.5% to 66%, 4,5,7 and was noted in four of our patients.…”

Section: Discussionsupporting

confidence: 80%

Scoliosis in patients with Friedreich’s ataxia

Smith

2012

The Journal of Bone and Joint Surgery. British volume

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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.Friedreich's ataxia (FA) is the most common progressive spinocerebellar degenerative disorder characterised by ataxia and was first described by Nikolaus Friedreich in the late 19th century.

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