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Friedreich's ataxia surgery

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Overview

Surgery is usually used for patients with either: Scoliosis, progressive equinovarus deformity, progressive severe dysphagia with endoscopic gastrostomy and progressive severe cardiac failure with cardiac transplantation.

Indications and Surgery

Surgery is usually used for patients with either:

  • Scoliosis[1][2]
    • The scoliosis progress in most children initially treated with a brace and finally, they required surgery.
  • Progressive equinovarus deformity[3][4]
    • Surgery may relieve pain and re-balance muscle forces across the foot of patients with Friedreich’s ataxia, helping the patient’s gait and preventing the progression or recurrence of the deformity.
  • Progressive severe dysphagia with endoscopic gastrostomy[5]
  • Progressive severe cardiac failure with cardiac transplantation[6]

References

  1. Tsirikos AI, Smith G (May 2012). “Scoliosis in patients with Friedreich’s ataxia”. J Bone Joint Surg Br. 94 (5): 684–9. doi:10.1302/0301-620X.94B5.28391. PMID 22529092.
  2. Cady RB, Bobechko WP (November 1984). “Incidence, natural history, and treatment of scoliosis in Friedreich’s ataxia”. J Pediatr Orthop. 4 (6): 673–6. PMID 6511892.
  3. Delatycki MB, Holian A, Corben L, Rawicki HB, Blackburn C, Hoare B, Toy M, Churchyard A (January 2005). “Surgery for equinovarus deformity in Friedreich’s ataxia improves mobility and independence”. Clin. Orthop. Relat. Res. (430): 138–41. PMID 15662315.
  4. Mancuso M, Orsucci D, Choub A, Siciliano G (September 2010). “Current and emerging treatment options in the management of Friedreich ataxia”. Neuropsychiatr Dis Treat. 6: 491–9. PMC 2938298. PMID 20856912.
  5. Bürk K (2017). “Friedreich Ataxia: current status and future prospects”. Cerebellum Ataxias. 4: 4. doi:10.1186/s40673-017-0062-x. PMC 5383992. PMID 28405347.
  6. McCormick A, Shinnick J, Schadt K, Rodriguez R, Addonizio L, Hirano M, Perlman S, Lin KY, Lynch DR (April 2017). “Cardiac transplantation in Friedreich Ataxia: Extended follow-up”. J. Neurol. Sci. 375: 471–473. doi:10.1016/j.jns.2017.01.027. PMID 28109580.

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