Anesth Pain Med.  2024 Jul;19(3):194-208. 10.17085/apm.24047.

Anesthetic considerations for joint replacement surgery in hemophilic arthropathy: a comprehensive review

• Ahmed HS
• Jayaram PR

• KMID: 2563559
• DOI: http://doi.org/10.17085/apm.24047

Abstract

Managing hemophilia in patients undergoing joint replacement surgery requires a comprehensive approach encompassing preoperative assessment, meticulous intraoperative care, and tailored postoperative management. Evaluation of joint integrity, bleeding history, and inhibitor presence guides surgical planning and hemostatic therapy selection to optimize outcomes. During surgery, careful attention to factor replacement, antibiotic prophylaxis, anesthetic techniques, and orthopedic strategies minimizes bleeding risk and enhances surgical success. Postoperatively, effective pain management, continued hemostatic therapy, and individualized rehabilitation programs are vital for facilitating recovery and preventing complications. Close monitoring for potential complications, such as periprosthetic joint infection and recurrent hemarthrosis, allows for prompt intervention when necessary. Overall, a collaborative approach involving hematologists, orthopedic surgeons, anesthesiologists, and rehabilitation specialists ensures comprehensive care tailored to the unique needs of patients with hemophilia undergoing joint replacement surgery, ultimately optimizing outcomes and improving quality of life. This holistic approach addresses the multifaceted challenges posed by hemophilia and joint replacement surgery, providing patients with the best possible chance for successful outcomes and long-term joint function. By integrating specialized expertise from multiple disciplines and implementing evidence-based strategies, healthcare providers can effectively manage hemophilia in the context of joint replacement surgery, mitigating risks and maximizing benefits for patients.

Keyword

Hemophilia A; Arthroplasty; Anesthesia; Hemostasis; Joint Diseases; Rehabilitation

Figure

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  Fig. 1. Decision tree for evaluating perioperative drug considerations of patients with hemophilia

Reference

1. Klatt EC, Kumar V. Robbins and cotran review of pathology. 4th ed. Philadelphia: Elsevier/Saunders;2015.

2. Pulles AE, Mastbergen SC, Schutgens RE, Lafeber FP, Van Vulpen LF. Pathophysiology of hemophilic arthropathy and potential targets for therapy. Pharmacol Res. 2017; 115:192–9.

3. Luck JV Jr, Silva M, Rodriguez-Merchan EC, Ghalambor N, Zahiri CA, Finn RS. Hemophilic arthropathy. J Am Acad Orthop Surg. 2004; 12:234–45.

4. Goto M, Takedani H, Nitta O, Kawama K. Joint function and arthropathy severity in patients with hemophilia. J Jpn Phys Ther Assoc. 2015; 18:15–22.

5. Adams JE, Reding MT. Hemophilic arthropathy of the elbow. Hand Clin. 2011; 27:151–63. v.

6. Wallny T, Lahaye L, Brackmann HH, Hess L, Seuser A, Kraft CN. Clinical and radiographic scores in haemophilic arthropathies: how well do these correlate to subjective pain status and daily activities? Haemophilia. 2002; 8:802–8.

7. Tripodi A, Santoro RC, Testa S, Molinari AC, Bernardini S, Golato M, et al. Position paper on laboratory testing for patients with haemophilia. A consensus document from SISET, AICE, SIBioC and SIPMeL. Blood Transfus. 2019; 17:229–36.

8. Bowyer AE, Gosselin RC. Factor VIII and factor IX activity measurements for hemophilia diagnosis and related treatments. Semin Thromb Hemost. 2023; 49:609–20.

9. Miller CH. Laboratory testing for factor VIII and IX inhibitors in haemophilia: A review. Haemophilia. 2018; 24:186–97.

10. Franchini M, Castaman G, Coppola A, Santoro C, Zanon E, Minno GD, et al. Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management. Blood Transfus. 2015; 13:498–513.

11. Meeks SL, Batsuli G. Hemophilia and inhibitors: current treatment options and potential new therapeutic approaches. Hematology Am Soc Hematol Educ Program. 2016; 2016:657–62.

12. Oldenburg J, Mahlangu JN, Kim B, Schmitt C, Callaghan MU, Young G, et al. Emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med. 2017; 377:809–18.

13. Holstein K, Le Quellec S, Klamroth R, Batorova A, Holme PA, Jiménez-Yuste V, et al. Immune tolerance induction in the era of emicizumab - still the first choice for patients with haemophilia A and inhibitors? Haemophilia. 2022; 28:215–22.

14. Chu WM, Ho HE, Wang JD, Chan WC, Liou YS, Ho WC, et al. Risk of major comorbidities among workers with hemophilia: A 14-year population-based study. Medicine (Baltimore). 2018; 97:e9803.

15. Soucie JM, Le B, Dupervil B, Poston JN. Prevalence of comorbid conditions among older males with haemophilia receiving care in haemophilia treatment centers in the United States. Haemophilia. 2022; 28:986–95.

16. Thachil J. Antiplatelet therapy - a summary for the general physicians. Clin Med (Lond). 2016; 16:152–60.

17. Juurlink DN. Antidepressants, antiplatelets and bleeding: one more thing to worry about? CMAJ. 2011; 183:1819–20.

18. Olas B. Dietary supplements with antiplatelet activity: a solution for everyone? Adv Nutr. 2018; 9:51–7.

19. Steiner M, Li W. Aged garlic extract, a modulator of cardiovascular risk factors: a dose-finding study on the effects of AGE on platelet functions. J Nutr. 2001; 131:980S–4S.

20. Harvey AL. Natural products in drug discovery. Drug Discov Today. 2008; 13:894–901.

21. Santoro RC, Molinari AC, Leotta M, Martini T. Isolated prolongation of activated partial thromboplastin time: not just bleeding risk! Medicina (Kaunas). 2023; 59:1169.

22. Whitton TP, Healy WJ. Clinical use and interpretation of thromboelastography. ATS Sch. 2023; 4:96–7.

23. Drotarova M, Zolkova J, Belakova KM, Brunclikova M, Skornova I, Stasko J, et al. Basic principles of rotational thromboelastometry (ROTEM^®) and the role of ROTEM-guided fibrinogen replacement therapy in the management of coagulopathies. Diagnostics (Basel). 2023; 13:3219.

24. Jacobs P. The surgeon, the anaesthetist, and the bleeding patient. Can the haematologist help? S Afr Med J. 1981; 59:419–21.

25. Malec L, Matino D. Targeting higher factor VIII levels for prophylaxis in haemophilia A: a narrative review. Haemophilia. 2023; 29:1419–29.

26. Delgado-Flores CJ, García-Gomero D, Salvador-Salvador S, Montes-Alvis J, Herrera-Cunti C, Taype-Rondan A. Effects of replacement therapies with clotting factors in patients with hemophilia: A systematic review and meta-analysis. PLoS One. 2022; 17:e0262273.

27. Ferreira AA, Bustamante-Teixeira MT, Leite IC, Corrêa CS, Rodrigues Dde O, da Cruz DT. Clinical and functional evaluation of the joint status of hemophiliac adults at a Brazilian blood center. Rev Bras Hematol Hemoter. 2013; 35:23–8.

28. Wang R, Wang Z, Gu Y, Zhang J, Wang P, Tong P, et al. Total knee arthroplasty in patients with haemophilic arthropathy is effective and safe according to the outcomes at a mid-term follow-up. J Orthop Traumatol. 2022; 23:31.

29. Knobe K, Berntorp E. Haemophilia and joint disease: pathophysiology, evaluation, and management. J Comorb. 2011; 1:51–9.

30. Gouw SC, Timmer MA, Srivastava A, De Kleijn P, Hilliard P, Peters M, et al. Measurement of joint health in persons with haemophilia: A systematic review of the measurement properties of haemophilia-specific instruments. Haemophilia. 2019; 25:e1–10.

31. Knöbl P. Prevention and management of bleeding episodes in patients with acquired hemophilia A. Drugs. 2018; 78:1861–72.

32. Chohan AS. Anesthetic considerations in orthopedic patients with or without trauma. Top Companion Anim Med. 2010; 25:107–19.

33. Matino D, Germini F, Chan AKC, Decker K, Iserman E, Chelle P, et al. Canadian clinical experience on switching from standard half-life recombinant factor VIII (rFVIII), octocog alfa, to extended half-life rFVIII, damoctocog alfa pegol, in persons with haemophilia A ≥ 12 years followed in a Comprehensive Hemophilia Care Program in Canada. Haemophilia. 2024; 30:345–54.

34. Trakymiene SS, Ingerslev J, Rageliene L. Utility of the Haemophilia Joint Health Score in study of episodically treated boys with severe haemophilia A and B in Lithuania. Haemophilia. 2010; 16:479–86.

35. Poonnoose PM, Manigandan C, Thomas R, Shyamkumar NK, Kavitha ML, Bhattacharji S, et al. Functional Independence Score in Haemophilia: a new performance-based instrument to measure disability. Haemophilia. 2005; 11:598–602.

36. Soliman M, Daruge P, Dertkigil SSJ, De Avila Fernandes E, Negrao JR, De Aguiar Vilela Mitraud S, et al. Imaging of haemophilic arthropathy in growing joints: pitfalls in ultrasound and MRI. Haemophilia. 2017; 23:660–72.

37. Cross S, Vaidya S, Fotiadis N. Hemophilic arthropathy: a review of imaging and staging. Semin Ultrasound CT MR. 2013; 34:516–24.

38. Prasetyo M, Moniqa R, Tulaar A, Prihartono J, Setiawan SI. Correlation between Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score and Hemophilia Joint Health Score (HJHS) in patients with hemophilic arthropathy. PLoS One. 2021; 16:e0248952.

39. Jiménez-Yuste V, Rodriguez-Merchan EC, Alvarez MT, Quintana M, Fernandez I, Hernandez-Navarro F. Controversies and challenges in elective orthopedic surgery in patients with hemophilia and inhibitors. Semin Hematol. 2008; 45(2 Suppl 1):S64–7.

40. De la Corte-Rodriguez H, Rodriguez-Merchan EC, Alvarez-Roman MT, Martin-Salces M, Martinoli C, Jimenez-Yuste V. The value of HEAD-US system in detecting subclinical abnormalities in joints of patients with hemophilia. Expert Rev Hematol. 2018; 11:253–61.

41. Rodriguez-Merchan EC. Cartilage damage in the haemophilic joints: pathophysiology, diagnosis and management. Blood Coagul Fibrinolysis. 2012; 23:179–83.

42. Brakel BA, Sussman MS, Majeed H, Teitel J, Man C, Rayner T, et al. T2 mapping magnetic resonance imaging of cartilage in hemophilia. Res Pract Thromb Haemost. 2023; 7:102182.

43. Cuesta-Barriuso R, Gómez-Conesa A, López-Pina JA. Physiotherapy treatment in patients with hemophilia and chronic ankle arthropathy: a systematic review. Rehabil Res Pract. 2013; 2013:305249.

44. De Kleijn P, Blamey G, Zourikian N, Dalzell R, Lobet S. Physiotherapy following elective orthopaedic procedures. Haemophilia. 2006; 12 Suppl 3:108–12.

45. Plut D, Kotnik BF, Pusnik L, Slak P, Snoj Z, Salapura V. Reliability of haemophilia early arthropathy detection with ultrasound (HEAD-US) in children: a comparative magnetic resonance imaging (MRI) study. Radiol Oncol. 2022; 56:471–8.

46. Benson G, Auerswald G, Dolan G, Duffy A, Hermans C, Ljung R, et al. Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management. Blood Transfus. 2018; 16:535–44.

47. Thornburg CD, Duncan NA. Treatment adherence in hemophilia. Patient Prefer Adherence. 2017; 11:1677–86.

48. Pitance V, Désage S, Lienhart A, Meunier S, Chamouard V. Haemophilia A patients' medication adherence to prophylaxis with efmoroctocog alfa. Haemophilia. 2021; 27:e368–75.

49. Srivastava A, Santagostino E, Dougall A, Kitchen S, Sutherland M, Pipe SW, WFH Guidelines for the Management of Hemophilia panelists and co-authors, et al. WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia. 2020; 26(Suppl 6):1–158.

50. Muluk V, Cohn SL, Whinney C. Perioperative medication management. SEFH Faquir [Internet]. 2021. Sep. [2021 May 4]. Available from: https://gruposdetrabajo.sefh.es/faquir/images/med-perioperatios/consenso/Perioperative_medication_management_-_UpToDate.pdf.

51. Clay BJ, Halasyamani L, Stucky ER, Greenwald JL, Williams MV. Results of a medication reconciliation survey from the 2006 Society of Hospital Medicine national meeting. J Hosp Med. 2008; 3:465–72.

52. Marcucci M, Painter TW, Conen D, Lomivorotov V, Sessler DI, Chan MTV, et al. Hypotension-avoidance versus hypertension-avoidance strategies in noncardiac surgery: an international randomized controlled trial. Ann Intern Med. 2023; 176:605–14.

53. Cruickshank RH, Morrison DA, Bamber PA, Nimmo WS. Effect of i.v. omeprazole on the pH and volume of gastric contents before surgery. Br J Anaesth. 1989; 63:536–40.

54. Canonico M, Oger E, Plu-Bureau G, Conard J, Meyer G, Lévesque H, Estrogen and Thromboembolism Risk (ESTHER) Study Group, et al. Hormone therapy and venous thromboembolism among postmenopausal women: impact of the route of estrogen administration and progestogens: the ESTHER study. Circulation. 2007; 115:840–5.

55. Van Hecken A, Schwartz JI, Depré M, De Lepeleire I, Dallob A, Tanaka W, et al. Comparative inhibitory activity of rofecoxib, meloxicam, diclofenac, ibuprofen, and naproxen on COX-2 versus COX-1 in healthy volunteers. J Clin Pharmacol. 2000; 40:1109–20.

56. Anderson JT, Barnes AJ, Stambough JB. The role of antiseptic irrigation solutions and topical antibiotics in total joint arthroplasty. J Surg Orthop Adv. 2021; 30:226–30.

57. Sahu S, Lata I, Singh S, Kumar M. Revisiting hemophilia management in acute medicine. J Emerg Trauma Shock. 2011; 4:292–8.

58. Peterson W, Tse B, Martin R, Fralick M, Sholzberg M. Evaluating hemostatic thresholds for neuraxial anesthesia in adults with hemorrhagic disorders and tendencies: A scoping review. Res Pract Thromb Haemost. 2021; 5:e12491.

59. Rodriguez-Patarroyo FA, Cuello N, Molloy R, Krebs V, Turan A, Piuzzi NS. A guide to regional analgesia for Total Knee Arthroplasty. EFORT Open Rev. 2021; 6:1181–92.

60. Memtsoudis SG, Cozowicz C, Bekeris J, Bekere D, Liu J, Soffin EM, et al. Anaesthetic care of patients undergoing primary hip and knee arthroplasty: consensus recommendations from the International Consensus on Anaesthesia-Related Outcomes after Surgery group (ICAROS) based on a systematic review and meta-analysis. Br J Anaesth. 2019; 123:269–87.

61. Moucha CS, Weiser MC, Levin EJ. Current strategies in anesthesia and analgesia for total knee arthroplasty. J Am Acad Orthop Surg. 2016; 24:60–73.

62. Dong CC, Dong SL, He FC. Comparison of adductor canal block and femoral nerve block for postoperative pain in total knee arthroplasty: a systematic review and meta-analysis. Medicine (Baltimore). 2016; 95:e2983.

63. Li D, Yang Z, Xie X, Zhao J, Kang P. Adductor canal block provides better performance after total knee arthroplasty compared with femoral nerve block: a systematic review and meta-analysis. Int Orthop. 2016; 40:925–33.

64. Seangleulur A, Vanasbodeekul P, Prapaitrakool S, Worathongchai S, Anothaisintawee T, McEvoy M, et al. The efficacy of local infiltration analgesia in the early postoperative period after total knee arthroplasty: A systematic review and meta-analysis. Eur J Anaesthesiol. 2016; 33:816–31.

65. Spangehl MJ, Clarke HD, Hentz JG, Misra L, Blocher JL, Seamans DP. The Chitranjan Ranawat Award: Periarticular injections and femoral & sciatic blocks provide similar pain relief after TKA: a randomized clinical trial. Clin Orthop Relat Res. 2015; 473:45–53.

66. Tateiwa T, Takahashi Y, Ishida T, Kubo K, Masaoka T, Shishido T, et al. Perioperative management of hemophilia patients receiving total hip and knee arthroplasty: a complication report of two cases. Ther Clin Risk Manag. 2015; 11:1383–9.

67. Rodríguez-Merchán EC. The role of orthopaedic surgery in haemophilia: current rationale, indications and results. EFORT Open Rev. 2019; 4:165–73.

68. Heeg M, Meyer K, Smid WM, Van Horn JR, Van der Meer J. Total knee and hip arthroplasty in haemophilic patients. Haemophilia. 1998; 4:747–51.

69. Rodriguez-Merchan EC. Total elbow arthroplasty in hemophilia: a high-risk surgical procedure. Expert Rev Hematol. 2023; 16:911–3.

70. Rodriguez-Merchan EC. Total ankle replacement in hemophilia. Cardiovasc Hematol Disord Drug Targets. 2020; 20:88–92.

71. Banerjee S, Issa K, Kapadia BH, Khanuja HS, Harwin SF, McInerney VK, et al. Intraoperative nonpharmacotherapeutic blood management strategies in total knee arthroplasty. J Knee Surg. 2013; 26:387–93.

72. Yoon SH, Bae J, Yoon S, Na KJ, Lee HJ. Correlation between pain intensity and quality of recovery after video-assisted thoracic surgery for lung cancer resection. J Pain Res. 2023; 16:3343–52.

73. Kaye AD, Urman RD, Rappaport Y, Siddaiah H, Cornett EM, Belani K, et al. Multimodal analgesia as an essential part of enhanced recovery protocols in the ambulatory settings. J Anaesthesiol Clin Pharmacol. 2019; 35(Suppl 1):S40–5.

74. Benyamin R, Trescot AM, Datta S, Buenaventura R, Adlaka R, Sehgal N, et al. Opioid complications and side effects. Pain Physician. 2008; 11(2 Suppl):S105–20.

75. Goode VM, Morgan B, Muckler VC, Cary MP Jr, Zdeb CE, Zychowicz M. Multimodal pain management for major joint replacement surgery. Orthop Nurs. 2019; 38:150–6.

76. Stromer W, Pabinger I, Ay C, Crevenna R, Donnerer J, Feistritzer C, et al. Pain management in hemophilia: expert recommendations. Wien Klin Wochenschr. 2021; 133:104256.

77. Palkovic B, Marchenko V, Zuperku EJ, Stuth EAE, Stucke AG. Multi-level regulation of opioid-induced respiratory depression. Physiology (Bethesda). 2020; 35:391–404.

78. Arachchillage DRJ, Makris M. Choosing and using non-steroidal anti-inflammatory drugs in haemophilia. Haemophilia. 2016; 22:179–87.

79. Kleiboer B, Layer MA, Cafuir LA, Cuker A, Escobar M, Eyster ME, et al. Postoperative bleeding complications in patients with hemophilia undergoing major orthopedic surgery: A prospective multicenter observational study. J Thromb Haemost. 2022; 20:857–65.

80. Abrantes JA, Solms A, Garmann D, Nielsen EI, Jönsson S, Karlsson MO. Relationship between factor VIII activity, bleeds and individual characteristics in severe hemophilia A patients. Haematologica. 2020; 105:1443–53.

81. Pathak N, Munger AM, Charifa A, Laskin WB, Bisson E, Kupfer GM, et al. Total knee arthroplasty in hemophilia A. Arthroplast Today. 2020; 6:52–58.e1.

82. Iwakiri K, Ohta Y, Shibata Y, Minoda Y, Kobayashi A, Nakamura H. Initiating range of motion exercises within 24 hours following total knee arthroplasty affects the reduction of postoperative pain: A randomized controlled trial. Asia Pac J Sports Med Arthrosc Rehabil Technol. 2020; 21:11–6.

83. Wang F, Zhang QB, Zhou Y, Chen S, Huang PP, Liu Y, et al. The mechanisms and treatments of muscular pathological changes in immobilization-induced joint contracture: A literature review. Chin J Traumatol. 2019; 22:93–8.

84. Stephensen D. Rehabilitation of patients with haemophilia after orthopaedic surgery: a case study. Haemophilia. 2005; 11(Suppl 1):26–9.

85. Lobet S, Pendeville E, Dalzell R, Defalque A, Lambert C, Pothen D, et al. The role of physiotherapy after total knee arthroplasty in patients with haemophilia. Haemophilia. 2008; 14:989–98.

86. Rodriguez-Merchan EC. Total knee replacement in haemophilic arthropathy. J Bone Joint Surg Br. 2007; 89:186–8.

87. Mortazavi SMJ, Haghpanah B, Ebrahiminasab MM, Baghdadi T, Toogeh G. Functional outcome of total knee arthroplasty in patients with haemophilia. Haemophilia. 2016; 22:919–24.

88. Broderick CR, Herbert RD, Latimer J, Barnes C, Curtin JA, Mathieu E, et al. Association between physical activity and risk of bleeding in children with hemophilia. JAMA. 2012; 308:1452–9.

89. Diez-Escudero A, Hailer NP. The role of silver coating for arthroplasty components. Bone Joint J. 2021; 103-B:423–9.

90. Suleiman LI, Mesko DR, Nam D. Intraoperative considerations for treatment/prevention of prosthetic joint infection. Curr Rev Musculoskelet Med. 2018; 11:401–8.

91. Bosco J, Bookman J, Slover J, Edusei E, Levine B. Principles of antibiotic prophylaxis in total joint arthroplasty: current concepts. Instr Course Lect. 2016; 65:467–75.

92. Park JJ, Slover JD, Stuchin SA. Recurrent hemarthrosis in a hemophilic patient after revision total knee arthroplasty. Orthopedics. 2010; 33:771.

93. Frauchiger LH, Harstall R, Kajahn J, Anderson S, Eggli S. Bilateral total knee arthroplasty in a patient with hemophilia A, high inhibitor titre and aneurysma spurium of the popliteal artery. A case report. Swiss Med Wkly. 2010; 140:w13094.

94. Chung SW, Seo YJ, You CW, Chun TJ, Jung KJ, Kim JH. A combined ultrasonographic and conventional radiographic assessment of hemophilic arthropathy. Indian J Hematol Blood Transfus. 2017; 33:380–8.

95. Wu GL, Zhai JL, Feng B, Bian YY, Xu C, Weng XS. Total hip arthroplasty in hemophilia patients: a mid‐term to long‐term follow‐up. Orthop Surg. 2017; 9:359–64.

96. Cheung EV, Sperling JW, Cofield RH. Infection associated with hematoma formation after shoulder arthroplasty. Clin Orthop Relat Res. 2008; 466:1363–7.