Case report| Volume 20, ISSUE 3, P250-253, July 2011

Labor and delivery in a patient with hemophilia B


      Hemophilia B is a rare X-linked disorder that may cause dramatic bleeding. Women account for only 3.2% of those clinically affected. The X-linked inheritance frequently delays the diagnosis in women and may expose the patient to an increased risk of adverse events. There is limited experience with these patients during labor and delivery. A 28-year-old primiparous woman with hemophilia B (bleeding phenotype) delivered a male infant by an unplanned cesarean delivery under general anesthesia following treatment with factor IX and normalization of her coagulation parameters, guided by thromboelastography. Postpartum vaginal bleeding required transfusion of two units of packed red blood cells. Factor IX supplementation continued for one week. Once diagnosed with hemophilia B, a multidisciplinary approach and advanced antenatal planning can increase the likelihood of a safe delivery. Neuraxial approaches and cesarean delivery are recommended only after normalization of the coagulation profile. The male fetus of a hemophilia A or B patient requires special attention. Operative vaginal delivery and invasive fetal monitoring should be avoided. Thromboelastography is an excellent technique to assess parturients with bleeding disorders or peripartum hemorrhage and may be underused.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to International Journal of Obstetric Anesthesia
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Mannucci M.D.
        • Tuddenham E.G.D.F.
        Medical progress: the Hemophilias – from royal genes to gene therapy.
        N Engl J Med. 2001; 344: 1773-1779
        • Lyon M.F.
        Gene action in the X-chromosome of the mouse.
        Nature. 1961; 190: 372-373
        • Wadelius C.
        • Lindstedt M.
        • Pigg M.
        • Egberg N.
        • Petersson U.
        • Anvret M.
        Hemophilia B in a 46, XX female probably caused by nonrandom X inactivation.
        Clin Genet. 1993; 43: 1-4
        • Orstavik K.H.
        • Orstavik R.E.
        • Eiklid K.
        • Tranebjaerg L.
        Inheritance of skewed X chromosome inactivation.
        Am J Med Genet. 1996; 64: 31-34
        • Taylor S.A.M.
        • Deugau K.V.
        • Lillicrap D.P.
        Somatic mosaicism and female-to-female transmission in a kindred with hemophilia B (factor IX deficiency).
        Proc Natl Acad Sci. 1991; 88: 39-42
        • Costa J.M.
        • Vidaud D.
        • Laurendeau T.J.
        Somatic mosaicism and compound heterozygosity in female hemophilia B.
        Blood. 2000; 96: 1585-1587
      1. Centers for Disease Control and Prevention. Report on the Universal Data Collection Program (UDC) 2002;4:1–28.

        • James A.H.
        • Hoots K.
        The optimal mode of delivery for a haemophilia carrier expecting an affected infant is caesarean delivery.
        Haemophilia. 2010; 16: 420-424
        • Ljung R.
        The optimal mode of delivery for a haemophilia carrier expecting an affected infant is vaginal delivery.
        Haemophilia. 2010; 16: 415-419
        • Walfish M.
        • Neuman A.
        • Wlody D.
        Maternal haemorrhage.
        Br J Anaesth. 2009; 103: i47-i56
        • Chang J.
        • Elam-Evans L.D.
        • Berg C.J.
        Pregnancy-related mortality surveillance-United States, 1991–1999.
        MMWR Surveill Summ. 2003; 52: 1-8
        • Kadir R.A.
        • Aledort L.M.
        Obstetrical and gynaecological bleeding: a common presenting symptom.
        Clin Lab Haematol. 2000; 22: 12-16
        • Kadir R.A.
        Frequency of inherited bleeding disorders in women with menorrhagia.
        Lancet. 1998; 351: 485-489
        • Ragni M.V.
        • Bontempo F.A.
        • Cortese-Hassett A.L.
        von Willebrand disease and bleeding in women.
        Hemophilia. 1999; 5: 313-317
        • Yang M.Y.
        • Ragni M.V.
        Clinical manifestations and management of labor and delivery in women with factor IX deficiency.
        Hemophilia. 2004; 10: 483-490
        • Levy J.H.
        • Dutton R.P.
        • Hemphill 3rd, J.C.
        • et al.
        Multidisciplinary approach to the challenge of hemostasis.
        Anesth Analg. 2010; 110: 354-364
        • Choi S.
        • Brull R.
        Neuraxial techniques in obstetric and non-obstetric patients with common bleeding diatheses.
        Anesth Analg. 2009; 109: 648-660
        • Chi C.
        • Lee C.A.
        • England A.
        • Hingrani J.
        • Paintsil J.
        • Kadir R.A.
        Obstetric analgesia and anaesthesia in women with inherited bleeding disorders.
        Thromb Haemost. 2009; 101: 1104-1111
        • Reikvam H.
        • Steien E.
        • Hauge B.
        Transfus Apher Sci. 2009; 40: 119-123
        • Othman M.
        • Falcón B.J.
        • Kadir R.
        Global hemostasis in pregnancy: are we using thrombelastography to its full potential?.
        Semin Thromb Hemost. 2010; 36: 738-746
        • Davies J.R.
        • Fernando R.
        • Hallworth S.P.
        Hemostatic function in healthy pregnant and preeclamptic women: an assessment using the platelet function analyzer (PFA-100) and thrombelastograph.
        Anesth Analg. 2007; 104: 416-420
        • Wegner J.
        • Popovsky M.A.
        Clinical utility of thromboelastography: one size does not fit all.
        Semin Thromb Hemost. 2010; 36: 699-706
        • Girolami A.
        • Scandellari R.
        • Vettore S.
        • Girolami B.
        Associated prothrombotic conditions are probably responsible for the occurrence of thrombosis in almost all patients with congenital FVII deficiency. Critical review of the literature.
        J Thromb Thrombolysis. 2010; 30: 172-178
        • Pradhan S.M.
        • Key N.S.
        • Boggio L.
        • Pruthi R.
        Venous thrombosis prophylaxis in haemophilics undergoing major orthopedic surgery: a survey of haemophilia treatment centres.
        Haemophilia. 2009; 15: 1337-1338
        • Rattray B.
        • Nugent D.J.
        • Young G.
        Celecoxib in the treatment of haemopilic synovitis, target joints, and pain in adults and children with haemophilia.
        Haemophilia. 2006; 12: 514-517
        • Inwood M.J.
        • Killackey B.
        • Startup S.J.
        The use and safety of Ibuprofen in the hemophiliac.
        Blood. 1983; 61: 709-711