Document Type: EDITORIAL

Author

Department of Orthopaedic Surgery, La Paz University Hospital, Madrid, Spain

Abstract

Modern orthopedic surgery can improve the musculoskeletal problems of people with hemophilia. The most commonly affected joints are the knees, the ankles and the elbows. The most common orthopedic procedures that PWH undergo are the following: RS, arthroscopic synovectomy, arthroscopic joint debridement, ankle arthrodesis, total joint arthroplasty and removal of pseudotumors. Every surgical procedure must be performed with adequate clotting factor coverage, following the indications of the hematologist in charge. For PWH, routine pharmacological thromboprophylaxis is not indicated. In patients with inhibitors (acquired antibodies against the deficient coagulation factor) and for the surgical removal of pseudotumors, local fibrin glue is advised. The mean infection rate with TKA in PWH is 7%. Intraarticular TXA is advised to control surgical bleeding. LIA is also recommended to control postoperative pain after TKA.

Keywords

Main Subjects

1. Rodriguez-Merchan EC. Musculoskeletal complications
of hemophilia. HSS J. 2010; 6(1):37-42.
2. Rodriguez-Merchan EC. Musculo-skeletal
manifestations of haemophilia. Blood Rev. 2016;
30(5):401-9.
3. Zozulya N, Kessler CM, Klukowska A, von Depka
M, Hampton K, Hay CR, et al. Efficacy and safety of
Nuwiq® (human-cl rhFVIII) in patients with severe
haemophilia A undergoing surgical procedures.
Haemophilia. 2018; 24(1):70-6.
4. Escobar MA, Tehranchi R, Karim FA, Caliskan U,
Chowdary P, Colberg T, et al. Low-factor consumption
for major surgery in haemophilia B with long-acting
recombinant glycoPEGylated factor IX. Haemophilia.
2017; 23(1):67-76.
5. Castaman G. The role of recombinant activated
factor VII in the haematological management of
elective orthopedic surgery in haemophilia A
patients with inhibitors. Blood Transfus. 2017;
15(5):478-86.
6. Danielson H, Lassila R, Ylinen P, Yrjönen T. Total
joint replacement in inhibitor-positive haemophilia:
Long-term outcome analysis in fifteen patients.
World J Orthop. 2017; 8(10):777-84.
7. Mortazavi SM, Najafi A, Toogeh G. Total joint
replacement in haemophilia A patients with high
titre of inhibitor using a new brand recombinant
factor VIIa (Aryoseven(®) ). Haemophilia. 2016;
22(5):e451-3.
8. Rodriguez-Merchan EC. Management of hemophilic
arthropathy of the ankle. Cardiovasc Hematol Disord
Drug Targets. 2017; 17(2):111-8.
9. Takedani H, Hirose J, Minamoto F, Kubota M,
Kinkawa J, Noguchi M. Major orthopedic surgery
for a haemophilia patient with inhibitors using a
new bypassing agent. Haemophilia. 2016; 22(5):
e459-61.
10. Liddle AD, Rodriguez-Merchan EC. Evidence-based
management of the knee in hemophilia. JBJS Rev.
2017; 5(8):e12.
11. Rodriguez-Merchan EC, Valentino LA. Orthopedic
disorders of the knee in hemophilia: a current
concept review. World J Orthop. 2016; 7(6):370-5.
12. Rodriguez-Merchan EC, Romero-Garrido JA, Gomez-
Cardero P. Multimodal blood loss prevention
approach including intra-articular tranexamic acid
in primary total knee arthroplasty for patients
with severe haemophilia A. Haemophilia. 2016;
22(4):e318-20.
13. Rodriguez-Merchan EC. Single local infiltration
analgesia (LIA) aids early pain management after
total knee replacement (TKR): an evidence-based
review and commentary. HSS J. 2018; 14(1):47-9.
14. Moore MF, Tobase P, Allen DD. Meta-analysis:
outcomes of total knee arthroplasty in the
haemophilia population. Haemophilia. 2016;
22(4):e275-85.
15. Ernstbrunner L, Hingsammer A, Catanzaro S, Sutter
R, Brand B, Wieser K, et al. Long-term results of
total knee arthroplasty in haemophilic patients:
an 18-year follow-up. Knee Surg Sports Traumatol
Arthrosc. 2017; 25(11):3431-8.
16. Rodriguez-Merchan EC. Simultaneous bilateral total
knee arthroplasty in hemophilia: is it recommended?
Expert Rev Hematol. 2017; 10(10):847-51.
17. Mortazavi SMJ, Firoozabadi MA, Najafi A, Mansouri
P. Evaluation of outcomes of suction drainage in
patients with haemophilic arthropathy undergoing
total knee arthroplasty. Haemophilia. 2017;
23(4):e310-5.
18. Park YS, Shin WJ, Kim KI. Comparison of continuous
infusion versus bolus injection of factor concentrates
for blood management after total knee arthroplasty
in patients with hemophilia. BMC Musculoskelet
Disord. 2017; 18(1):356.
19. Yasui T, Hirose J, Ono K, Takedani H. Arthroscopic
debridement for advanced haemophilic ankle
arthropathy. Haemophilia. 2017; 23(5):e479-81.
20. Rodriguez-Merchan EC. Joint distraction in advanced
haemophilic ankle arthropathy. Haemophilia. 2016;
22(4):e301-4.
21. Brkljac M, Shah S, Hay C, Rodriguez-Merchan EC.
Hindfoot fusion in haemophilic arthropathy: 6-year
mean follow-up of 41 procedures performed in 28
adult patients. Haemophilia. 2016; 22(2):e87-98.
22. de l’Escalopier N, Badina A, Padovani JP, Harroche
A, Frenzel L, Wicart P, et al. Long-term results of
ankle arthrodesis in children and adolescents with
haemophilia. Int Orthop. 2017; 41(8):1579-84.
23. Preis M, Bailey T, Jacxsens M, Barg A. Total
ankle replacement in patients with haemophilic
arthropathy: primary arthroplasty and conversion
of painful ankle arthrodesis to arthroplasty.
Haemophilia. 2017; 23(4):e301-9.
24. Strauss AC, Rommelspacher Y, Nouri B, Bornemann
R, Wimmer MD, Oldenburg J, et al. Long-term
outcome of total hip arthroplasty in patients with
haemophilia. Haemophilia. 2017; 23(1):129-34.
25. Ernstbrunner L, Hingsammer A, Imam MA, Sutter R,
Brand B, Meyer DC, et al. Long-term results of total
elbow arthroplasty in patients with hemophilia. J
Shoulder Elbow Surg. 2018; 27(1):126-32.
26. Zhai J, Weng X, Zhang B, Liu Y, Gao P, Bian YY. Surgical
treatment for hemophilic pseudotumor: twentythree
cases with an average follow-up of 5 years. J
Bone Joint Surg Am. 2017; 99(11):947-53.
27. Rodriguez-Merchan EC. Fibrin glue for local
haemostasis in haemophilia surgery. Hosp Pract (1995). 2017; 45(5):187-91.

28. Ahmed A, Kozek-Langenecker S, Mullier F, Pavord
S, Hermans C; ESA VTE Guidelines Task Force.
European guidelines on perioperative venous
thromboembolism prophylaxis: Patients with
preexisting coagulation disorders and after severe
perioperative bleeding. Eur J Anaesthesiol. 2018;
35(2):96-107.
29. Kachooei AR, Badiei Z, Zandinezhad ME,
Ebrahimzadeh MH, Mazloumi SM, Omidi-Kashani F,
et al. Influencing factors on the functional level of
haemophilic patients assessed by FISH. Haemophilia.
2014; 20(2):185-9.