title: Surgery for equinovarus deformity in Friedreich's ataxia improves mobility and independence.
abstract: Friedreich's ataxia results in morbidity because of many factors; progressive equinovarus deformity is one of these. We studied the risk factors and incidence of this deformity. We sought to assess whether surgical management of fixed equinovarus deformity leads to functional improvement. Thirty-six patients with Friedrich's ataxia were assessed for this deformity. These patients were treated by splinting, botulinum toxin Type A injection, and surgery, as indicated by the severity, followed by an ongoing rehabilitation program. The effect of surgery was assessed using subscales of the Barthel index and functional independence measure. Severe foot deformities in which either surgery or botulinum toxin injection was recommended correlated with current age, years since disease onset, and years that the patient required a wheelchair for mobility, but not with the GAA repeat size or age at disease onset. Function and mobility were improved after surgery compared with a similar period before surgery. Three of seven patients who had surgery had significant complications. Aggressive management of foot deformities should be considered, and active measures to prevent permanent foot deformities should be pursued to maximize quality of life and independence of patients with Friedreich's ataxia.
  
  
  METHODS
  Therapeutic study, Level IV (case series-no, or historical control group).
authors.affiliation: Bruce Lefroy Centre for Genetic Health Research, Genetic Health Services Victoria, Murdoch Childrens Research Institute, Royal Children's Hospital, Victoria, Australia. martin.delatycki@ghsv.org.au
authors.forename: Martin B
authors.initials: MB
authors.lastname: Delatycki
authors.forename: Annette
authors.initials: A
authors.lastname: Holian
authors.forename: Louise
authors.initials: L
authors.lastname: Corben
authors.forename: H Barry
authors.initials: HB
authors.lastname: Rawicki
authors.forename: Christine
authors.initials: C
authors.lastname: Blackburn
authors.forename: Brian
authors.initials: B
authors.lastname: Hoare
authors.forename: Melanie
authors.initials: M
authors.lastname: Toy
authors.forename: Andrew
authors.initials: A
authors.lastname: Churchyard
mesh_terms: D000203:Activities of Daily Living; D019274:Botulinum Toxins, Type A / Q000627:therapeutic use; D004863:Equinus Deformity / Q000188:drug therapy / Q000209:etiology* / Q000601:surgery*; D005621:Friedreich Ataxia / Q000150:complications*; D006801:Humans; D017060:Patient Satisfaction; D011446:Prospective Studies; D011788:Quality of Life; D020127:Recovery of Function; D012307:Risk Factors; D013165:Splints; D016896:Treatment Outcome
chemical_list: D019274:Botulinum Toxins, Type A