Orthopedic Surgery for Friedreich Ataxia Guidelines

Updated: Nov 22, 2022
  • Author: Stephen Kishner, MD, MHA; Chief Editor: Vinod K Panchbhavi, MD, FACS, FAOA, FABOS, FAAOS  more...
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Guidelines for Management of Orthopedic Issues in Friedreich Ataxia

In November 2022, guidelines for the clinical management of Friedreich ataxia (FA) were published by an expert panel using the Grading of Recommendations Assessment and Evaluation (GRADE) framework for rare diseases developed by the RARE-Bestpractices Working Group. [3] The following recommendations and suggestions were made regarding orthopedic issues (ie, scoliosis and foot deformity) in patients with FA:

  • Spinal examination must be part of the multidisciplinary approach for individuals with FA and should be performed regularly
  • Individuals with FA who have a spinal curve between 20° and 40° and/or are between the ages of 10 and 16 years should be observed for curve progression; bracing may not reduce or stop curve progression, but it may be valuable in delaying surgical correction in young children
  • Patients with scoliosis >40° may be considered appropriate for surgical correction
  • Delay of surgical intervention should be considered in ambulant individuals with FA
  • For all FA patients being considered for scoliosis surgery, extensive preoperative evaluation and planning regarding cardiac and pulmonary function are required
  • It is suggested that surgery not be used over conservative therapy for individuals with scoliosis and FA
  • Age should be considered when scoliosis surgery is contemplated for individuals with FA
  • Stratification according to cardiac status is recommended when scoliosis surgery is contemplated for individuals with FA
  • Orthopedic surgery is conditionally recommended for FA patients with foot issues if it is considered that such surgery would assist in foot alignment to facilitate mobility and/or safe and effective transfers
  • Orthotics are conditionally recommended for FA patients with foot issues 
  • It is not possible to recommend either physiotherapy or no physiotherapy for FA patients with foot issues