Clinical Orthopaedics and Related Research®

, Volume 469, Issue 5, pp 1427–1435 | Cite as

Total Ankle Arthroplasty in Patients with Hereditary Hemochromatosis

  • Alexej Barg
  • Andreas Elsner
  • Daniel Hefti
  • Beat Hintermann
Clinical Research



More than half of patients with hereditary hemochromatosis (HH) have painful arthritis, often including hindfoot osteoarthritis. Total ankle arthroplasty (TAA) is increasingly recommended for patients with painful ankle osteoarthritis. However, the pain relief and function experienced by patients continues to be debated particularly as compared with ankle fusion.


We asked whether (1) the complication rates were low; (2) the components were stable; (3) the patients achieved pain relief; and (4) the patients had satisfactory midterm function, ROM, and quality of life.

Patients and Methods

We retrospectively reviewed all 16 prospectively followed patients (21 implants) with HH who underwent ankle arthroplasty. They had an average age of 59.5 years at the time of surgery. We obtained a visual analog scale for pain, the SF-36, and the American Orthopaedic Foot and Ankle Society (AOFAS) hindfoot score. Component stability was assessed using weightbearing radiographs. The minimum followup was 3.1 years (average, 5.3 years; range, 3.1–8.6 years).


Postoperatively, one patient had débridement of a painful cyst on the tibial side and one patient had a subfibular débridement with a lateral ligament reconstruction. The tibial and talar components were stable in all ankles. The average pain score decreased from 6.7 (range, 3–10) to 1.9 (range, 0–4). All eight categories of SF-36 score showed improvement. The hindfoot score increased from 46 (range, 22–67) to 84 (range, 74–94).


Our data suggest TAA in patients with ankle osteoarthritis secondary to HH is associated with a low risk of postoperative complications and produces pain relief and good function.

Level of Evidence

Level IV, therapeutic case series. See Guidelines for Authors for a complete description of levels of evidence.


  1. 1.
    Allen KJ, Gurrin LC, Constantine CC, Osborne NJ, Delatycki MB, Nicoll AJ, McLaren CE, Bahlo M, Nisselle AE, Vulpe CD, Anderson GJ, Southey MC, Giles GG, English DR, Hopper JL, Olynyk JK, Powell LW, Gertig DM. Iron-overload-related disease in HFE hereditary hemochromatosis. N Engl J Med. 2008; 358:221–230.PubMedCrossRefGoogle Scholar
  2. 2.
    Anderson T, Montgomery F, Carlsson A. Uncemented STAR total ankle prostheses: three to eight-year follow-up of fifty-one consecutive ankles. J Bone Joint Surg Am. 2003;85:1321–1329.PubMedGoogle Scholar
  3. 3.
    Buechel FF Sr, Buechel FF Jr, Pappas MJ. Eighteen-year evaluation of cementless meniscal bearing total ankle replacements. Instr Course Lect. 2002;51:143–151.PubMedGoogle Scholar
  4. 4.
    Chou LB, Coughlin MT, Hansen S Jr, Haskell A, Lundeen G, Saltzman CL, Mann RA. Osteoarthritis of the ankle: the role of arthroplasty. J Am Acad Orthop Surg. 2008;16:249–259.PubMedGoogle Scholar
  5. 5.
    Coester LM, Saltzman CL, Leupold J, Pontarelli W. Long-term results following ankle arthrodesis for post-traumatic arthritis. J Bone Joint Surg Am. 2001;83:219–228.PubMedGoogle Scholar
  6. 6.
    Davies MB, Saxby T. Ankle arthropathy of hemochromatosis: a case series and review of the literature. Foot Ankle Int. 2006;27:902–906.PubMedGoogle Scholar
  7. 7.
    Fleiss JL. Statistical Methods for Rates and Proportions. Ed 2. New York, NY: John Wiley and Sons, 1981.Google Scholar
  8. 8.
    Fuchs S, Sandmann C, Skwara A, Chylarecki C. Quality of life 20 years after arthrodesis of the ankle: a study of adjacent joints. J Bone Joint Surg Br. 2003;85:994–998.PubMedCrossRefGoogle Scholar
  9. 9.
    Glazebrook M, Daniels T, Younger A, Foote CJ, Penner M, Wing K, Lau J, Leighton R, Dunbar M. Comparison of health-related quality of life between patients with end-stage ankle and hip arthrosis. J Bone Joint Surg Am. 2008;90:499–505.PubMedCrossRefGoogle Scholar
  10. 10.
    Gougoulias N, Khanna A, Maffulli N. How successful are current ankle replacements?: a systematic review of the literature. Clin Orthop Relat Res. 2010;468:199–208.PubMedCrossRefGoogle Scholar
  11. 11.
    Haddad SL, Coetzee JC, Estok R, Fahrbach K, Banel D, Nalysnyk L. Intermediate and long-term outcomes of total ankle arthroplasty and ankle arthrodesis: a systematic review of the literature. J Bone Joint Surg Am. 2007;89:1899–1905.PubMedCrossRefGoogle Scholar
  12. 12.
    Hintermann B, Valderrabano V, Dereymaeker G, Dick W. The HINTEGRA ankle: rationale and short-term results of 122 consecutive ankles. Clin Orthop Relat Res. 2004;424:57–68.PubMedCrossRefGoogle Scholar
  13. 13.
    Huskisson EC. Measurement of pain. Lancet. 1974; 2:1127–1131.PubMedCrossRefGoogle Scholar
  14. 14.
    Jacki SH, Uhl M, Adler CP, Peter HH, von Kempis J. Predominant ankle arthropathy in hereditary haemochromatosis. Rheumatology (Oxford). 1999;38:378–379.CrossRefGoogle Scholar
  15. 15.
    Karantana A, Martin Geoghegan J, Shandil M, Dhar S. Simultaneous bilateral total ankle replacement using the S.T.A.R.: a case series. Foot Ankle Int. 2010;31:86–89.PubMedCrossRefGoogle Scholar
  16. 16.
    Kim BS, Choi WJ, Kim YS, Lee JW. Total ankle replacement in moderate to severe varus deformity of the ankle. J Bone Joint Surg Br. 2009;91:1183–1190.PubMedCrossRefGoogle Scholar
  17. 17.
    Kitaoka HB, Alexander IJ, Adelaar RS, Nunley JA, Myerson MS, Sanders M. Clinical rating systems for the ankle-hindfoot, midfoot, hallux, and lesser toes. Foot Ankle Int. 1994;15:349–353.PubMedGoogle Scholar
  18. 18.
    Kopp FJ, Patel MM, Deland JT, O’Malley MJ. Total ankle arthroplasty with the Agility prosthesis: clinical and radiographic evaluation. Foot Ankle Int. 2006;27:97–103.PubMedGoogle Scholar
  19. 19.
    Lance EM, Paval A, Fries I, Larsen I, Patterson RL Jr. Arthrodesis of the ankle joint: a follow-up study. Clin Orthop Relat Res. 1979;142:146–158.PubMedGoogle Scholar
  20. 20.
    Lee KB, Cho SG, Hur CI, Yoon TR. Perioperative complications of HINTEGRA total ankle replacement: our initial 50 cases. Foot Ankle Int. 2008;29:978–984.PubMedCrossRefGoogle Scholar
  21. 21.
    Lynch AF, Bourne RB, Rorabeck CH. The long-term results of ankle arthrodesis. J Bone Joint Surg Br. 1988;70:113–116.PubMedGoogle Scholar
  22. 22.
    Mazur JM, Schwartz E, Simon SR. Ankle arthrodesis. Long-term follow-up with gait analysis. J Bone Joint Surg Am. 1979;61:964–975.PubMedGoogle Scholar
  23. 23.
    McCurdie I, Perry JD. Haemochromatosis and exercise related joint pains. BMJ. 1999;318:449–451.PubMedGoogle Scholar
  24. 24.
    McDonnell SM, Preston BL, Jewell SA, Barton JC, Edwards CQ, Adams PC, Yip R. A survey of 2,851 patients with hemochromatosis: symptoms and response to treatment. Am J Med. 1999;106:619–624.PubMedCrossRefGoogle Scholar
  25. 25.
    McLaren GD, McLaren CE, Adams PC, Barton JC, Reboussin DM, Gordeuk VR, Acton RT, Harris EL, Speechley MR, Sholinsky P, Dawkins FW, Snively BM, Vogt TM, Eckfeldt JH; Hemochromatosis and Iron Overload Screen (HEIRS) Study Research Investigators. Clinical manifestations of hemochromatosis in HFE C282Y homozygotes identified by screening. Can J Gastroenterol. 2008;22:923–930.PubMedGoogle Scholar
  26. 26.
    Morgan CD, Henke JA, Bailey RW, Kaufer H. Long-term results of tibiotalar arthrodesis. J Bone Joint Surg Am. 1985;67:546–550.PubMedGoogle Scholar
  27. 27.
    Myerson MS, Mroczek K. Perioperative complications of total ankle arthroplasty. Foot Ankle Int. 2003;24:17–21.PubMedGoogle Scholar
  28. 28.
    Nihal A, Gellman RE, Embil JM, Trepman E. Ankle arthrodesis. Foot Ankle Surg. 2008;14:1–10.PubMedCrossRefGoogle Scholar
  29. 29.
    Pagenstert GI, Barg A, Leumann AG, Rasch H, Muller-Brand J, Hintermann B, Valderrabano V. SPECT-CT imaging in degenerative joint disease of the foot and ankle. J Bone Joint Surg Br. 2009;91:1191–1196.PubMedCrossRefGoogle Scholar
  30. 30.
    Rihl M, Kellner H. [Arthropathy of hereditary hemochromatosis] [in German]. Z Rheumatol. 2004;63:22–29.PubMedCrossRefGoogle Scholar
  31. 31.
    Saltzman CL, Amendola A, Anderson R, Coetzee JC, Gall RJ, Haddad SL, Herbst S, Lian G, Sanders RW, Scioli M, Younger AS. Surgeon training and complications in total ankle arthroplasty. Foot Ankle Int. 2003;24:514–518.PubMedGoogle Scholar
  32. 32.
    Saltzman CL, el-Khoury GY. The hindfoot alignment view. Foot Ankle Int. 1995;16:572–576.PubMedGoogle Scholar
  33. 33.
    Saltzman CL, Mann RA, Ahrens JE, Amendola A, Anderson RB, Berlet GC, Brodsky JW, Chou LB, Clanton TO, Deland JT, Deorio JK, Horton GA, Lee TH, Mann JA, Nunley JA, Thordarson DB, Walling AK, Wapner KL, Coughlin MJ. Prospective controlled trial of STAR total ankle replacement versus ankle fusion: initial results. Foot Ankle Int. 2009;30:579–596.PubMedCrossRefGoogle Scholar
  34. 34.
    Schmid H, Struppler C, Braun GS, Kellner W, Kellner H. Ankle and hindfoot arthropathy in hereditary hemochromatosis. J Rheumatol. 2003;30:196–199.PubMedGoogle Scholar
  35. 35.
    Schutte BG, Louwerens JW. Short-term results of our first 49 Scandanavian total ankle replacements (STAR). Foot Ankle Int. 2008;29:124–127.PubMedCrossRefGoogle Scholar
  36. 36.
    SooHoo NF, Zingmond DS, Ko CY. Comparison of reoperation rates following ankle arthrodesis and total ankle arthroplasty. J Bone Joint Surg Am. 2007;89:2143–2149.PubMedCrossRefGoogle Scholar
  37. 37.
    Valderrabano V, Hintermann B, Dick W. Scandinavian total ankle replacement: a 3.7-year average followup of 65 patients. Clin Orthop Relat Res. 2004;424:47–56.PubMedCrossRefGoogle Scholar
  38. 38.
    Valderrabano V, Pagenstert G, Horisberger M, Knupp M, Hintermann B. Sports and recreation activity of ankle arthritis patients before and after total ankle replacement. Am J Sports Med. 2006;34:993–999.PubMedCrossRefGoogle Scholar
  39. 39.
    Ware JE Jr, Sherbourne CD. The MOS 36-item short-form health survey (SF-36): I. Conceptual framework and item selection. Med Care. 1992;30:473–483.PubMedCrossRefGoogle Scholar
  40. 40.
    Wood PL, Deakin S. Total ankle replacement: the results in 200 ankles. J Bone Joint Surg Br. 2003;85:334–341.PubMedCrossRefGoogle Scholar

Copyright information

© The Association of Bone and Joint Surgeons® 2010

Authors and Affiliations

  • Alexej Barg
    • 1
  • Andreas Elsner
    • 1
  • Daniel Hefti
    • 1
  • Beat Hintermann
    • 1
  1. 1.Clinic of Orthopaedic SurgeryKantonsspital LiestalLiestalSwitzerland

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