Known Bleeding Disorders for Surgery

  • Miguel A. EscobarEmail author
  • Trinh Nguyen


Persons with congenital bleeding disorders pose a surgical challenge as hemostasis is already difficult without the added iatrogenic stress of surgery. For some bleeding disorders, there are therapeutic options including plasma-derived blood products and even recombinant factor products that are available. These may be administered pre- and post-op to prevent bleeding complications. Unfortunately for some rare bleeding disorders, there are no commercially available plasma-derived concentrates or recombinant products to administer pre- or postoperatively to prevent hemorrhagic sequelae. Fresh frozen plasma or cryoprecipitate becomes the only available option for surgical prophylaxis. A non-transfusional alternative such as desmopressin may be used with/without replacement factor to maintain hemostasis, depending on the individual response to desmopressin. Antifibrinolytics are another option as monotherapy or adjunctive therapy to prevent bleeding diathesis. The following discussion serves as a guide to the perioperative management of individuals with congenital bleeding disorders based on their diagnosis, type of surgery, and commercially available products.


Congenital bleeding disorder Hemophilia Von Willebrand disease Fibrinogen Factor II Factor V Factor VII Factor VIII Factor IX Factor X Factor XI Factor XIII Surgery Prophylaxis Plasma-derived concentrates Recombinant factor FFP Cryoprecipitate Desmopressin Aminocaproic acid Tranexamic acid Fibrin 


  1. 1.
    Roseff SD, Eder A, Punzalan R, Sloan S, Strauss R, Wong ECC, et al., editors. Pediatric transfusion: a physician’s handbook. 3rd ed. Bethesda: AABB; 2009.Google Scholar
  2. 2.
    Rizza CR. Inhibitors of fibrinolysis in the treatment of haemophilia. J Clin Pathol Suppl (R Coll Pathol). 1980;14:50–4.CrossRefGoogle Scholar
  3. 3.
    Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, Montgomery RR, Ortel TL, et al. Rick ME, Sadler JE, Weinstein M, Yawn BP. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia. 2008;14(2):171–232. doi: 10.1111/j.1365-2516.2007.01643.x.CrossRefPubMedGoogle Scholar
  4. 4.
    Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A, et al. Treatment Guidelines Working Group on Behalf of The World Federation Of Hemophilia. Guidelines for the management of hemophilia. Haemophilia. 2013;19(1):e1–47. doi: 10.1111/j.1365-2516.2012.02909.x. Epub 2012 Jul 6.CrossRefPubMedGoogle Scholar
  5. 5.
    Mannucci PM. Treatment of von Willebrand’s disease. N Engl J Med. 2004;351(7):683–94.CrossRefPubMedGoogle Scholar
  6. 6.
    Escobar MA. Products used to treat haemophilia: dosing. In: Lee C, Hoots K, Berntorp E, editors. Textbook of haemophilia. 3rd ed. Oxford: Blackwell; 2014.Google Scholar
  7. 7.
    Rocino A, Coppola A, Franchini M, Castaman G, Santoro C, Zanon E, et al. Principles of treatment and update of recommendations for the management of haemophilia and congenital bleeding disorders in Italy. Blood Transfus. 2014;12(4):575–98. doi: 10.2450/2014.0223-14.PubMedPubMedCentralGoogle Scholar
  8. 8.
    Rickard KA. Guidelines for therapy and optimal dosages of coagulation factors for treatment of bleeding and surgery in haemophilia. Haemophilia. 1995;1(S1):8–13.CrossRefPubMedGoogle Scholar
  9. 9.
    Batorova A, Martinowitz U. Intermittent injections vs. continuous infusion of factor VIII in haemophilia patients undergoing major surgery. Br J Haematol. 2000;110(3):715–20.CrossRefPubMedGoogle Scholar
  10. 10.
    Auerswald G, Bade A, Haubold K, Overberg D, Masurat S, Moorthi C. No inhibitor development after continuous infusion of factor concentrates in subjects with bleeding disorders undergoing surgery: a prospective study. Haemophilia. 2013;19(3):438–44. doi: 10.1111/hae.12075. Epub 2012 Dec 20.CrossRefPubMedGoogle Scholar
  11. 11.
    Escobar MA, Maahs J, Hellman E, Donkin J, Forsyth A, Hroma N, et al. Best Practices for the multidisciplinary management of patients with hemophilia with inhibitors undergoing surgery in the United States. Haemophilia. 2012;18(6):971–81.CrossRefPubMedGoogle Scholar
  12. 12.
    Obergfell A, Auvinen MK, Mathew P. Recombinant activated factor VII for haemophilia patients with inhibitors undergoing orthopaedic surgery: a review of the literature. Haemophilia. 2008;14(2):233–41. Epub 2007 Dec 12.CrossRefPubMedGoogle Scholar
  13. 13.
    Teitel JM, Carcao M, Lillicrap D, Mulder K, Rivard GE, St-Louis J, et al. Orthopaedic surgery in haemophilia patients with inhibitors: a practical guide to haemostatic, surgical and rehabilitative care. Haemophilia. 2009;15(1):227–39. doi: 10.1111/j.1365-2516.2008.01840.x. Epub 2008 Aug 25.CrossRefPubMedGoogle Scholar
  14. 14.
    Giangrande PL, Wilde JT, Madan B, Ludlam CA, Tuddenham EG, Goddard NJ, et al. Consensus protocol for the use of recombinant activated factor VII [eptacog alfa (activated); NovoSeven] in elective orthopaedic surgery in haemophilic patients with inhibitors. Haemophilia. 2009;15(2):501–8. doi: 10.1111/j.1365-2516.2008.01952.x. Epub 2009 Feb 1.CrossRefPubMedGoogle Scholar
  15. 15.
    Escobar MA, Key N. Hemophilia A and Hemophilia B. In: Kaushansky K, Lichtman MA, Beutler E, Kipps TJ, Seligsohn U, Prchal JT, editors. Williams hematology. 9th ed. New York: McGraw Hill; 2016.Google Scholar
  16. 16.
    Brooker M. Registry of clotting factor concentrates [Internet]. Montreal, Quebec, Canada: World Federation of Hemophilia; 1998. [updated May 2012; cited 2013 August 28], Available from
  17. 17.
    Kreuz W, Meili E, Peter-Salonen K, Haertel S, Devay J, Krzensk U, Egbring R. Efficacy and tolerability of a pasteurised human fibrinogen concentrate in patients with congenital fibrinogen deficiency. Transfus Apher Sci. 2005;32(3):247–53.CrossRefPubMedGoogle Scholar
  18. 18.
    Mannucci PM, Duga S, Peyvandi F. Recessively inherited coagulation disorders. Blood. 2004;104(5):1243–52. Epub 2004 May 11.CrossRefPubMedGoogle Scholar
  19. 19.
    Stanworth SJ. The evidence-based use of FFP and cryoprecipitate for abnormalities of coagulation tests and clinical coagulopathy. Hematology Am Soc Hematol Educ Program. 2007;179–86.Google Scholar
  20. 20.
    Escobar MA, Roberts HR. Less common congenital disorders of hemostasis. In: Kitchens CS et al., editors. Consultative hemostasis and thrombosis. 3rd ed. Philadelphia: W.B. Saunders; 2013.Google Scholar
  21. 21.
    Bolton-Maggs PH, Perry DJ, Chalmers EA, Parapia LA, Wilde JT, Williams MD, et al. The rare coagulation disorders—review with guidelines for management from the United Kingdom Haemophilia Centre Doctors’ Organisation. Haemophilia. 2004;10(5):593–628.CrossRefPubMedGoogle Scholar
  22. 22.
    Roberts HR, Escobar MA. Other clotting factor deficiencies. In: Hoffman R et al., editors. Hematology: basic principles and practice. 4th ed. Philadelphia: Churchill Livingstone; 2005.Google Scholar
  23. 23.
    Di Paola J, Nugent D, Young G. Current therapy for rare factor deficiencies. Haemophilia. 2001;7 Suppl 1:16–22.CrossRefPubMedGoogle Scholar
  24. 24.
    Huang JN, Koerper MA. Factor V deficiency: a concise review. Haemophilia. 2008;14(6):1164–9. doi: 10.1111/j.1365-2516.2008.01785.x.CrossRefPubMedGoogle Scholar
  25. 25.
    Mariani G, Dolce A, Batorova A, Auerswald G, Schved JF, Siragusa S, et al. STER and the International Factor VII Deficiency Study Groups. Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER. Br J Haematol. 2011;152(3):340–6. doi: 10.1111/j.1365-2141.2010.08287.x. Epub 2010 Dec 16.CrossRefPubMedGoogle Scholar
  26. 26.
    Barnett JM, Demel KC, Mega AE, Butera JN, Sweeney JD. Lack of bleeding in patients with severe factor VII deficiency. Am J Hematol. 2005;78(2):134–7.CrossRefPubMedGoogle Scholar
  27. 27.
    Escobar M, Austin S, Auerswald G, Millar C, MacDonald S, Norton M, Aldwinckle T. Safety and efficacy of a new high purity factor X concentrate in subjects with factor X deficiency undergoing surgery. In: XXXI International Congress of the World Federation of Hemophilia, Melbourne, Australia: May 2014 (abstract).Google Scholar
  28. 28.
    Coagadex [package insert]. Hertfordshire: Bio Products Laboratory; 2015.Google Scholar
  29. 29.
    Brown DL, Kouides PA. Diagnosis and treatment of inherited factor X deficiency. Haemophilia. 2008;14(6):1176–82. doi: 10.1111/j.1365-2516.2008.01856.x.CrossRefPubMedGoogle Scholar
  30. 30.
    Berliner S, Horowitz I, Martinowitz U, Brenner B. Seligsohn. Dental Surgery in patients with severe factor XI deficiency without plasma replacement. Blood Coagul Fibrinolysis. 1992;3(4):465–8.CrossRefPubMedGoogle Scholar
  31. 31.
    Bolton-Maggs PHB. The rare coagulation disorders [Internet]. Montreal, QC: World federation of hemophilia; 2006.
  32. 32.
    Nugent D. Corifact™/Fibrogammin P in the prophylactic treatment of hereditary factor XIII deficiency: results of a prospective, multicenter, open-label study. Thromb Res. 2012;130 Suppl 2:S12–4. doi: 10.1016/S0049-3848(13)70005-7.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of HematologyGulf States Hemophilia and Thrombophilia Center, University of Texas Health Center and McGovern Medical SchoolHoustonUSA
  2. 2.Department of PediatricsMD Anderson Children’s Cancer Center and Gulf States Hemophilia and Thrombophilia Treatment Center, The University of Texas-Health Science Center at HoustonHoustonUSA

Personalised recommendations