Obstetric Management of Adolescents with Bleeding Disorders

Published:October 11, 2010DOI:


      Adolescents with bleeding disorders who become pregnant must contend with the dual challenges of their bleeding disorder and their pregnancy. Adolescents are more likely to terminate a pregnancy than adult women, and when they do carry a pregnancy, they are more likely to deliver prematurely. Otherwise, they are at risk for the same complications that adult women with bleeding disorders experience, particularly bleeding complications postpartum. Since one half to two thirds of adolescent pregnancies are unplanned, issues related to reproduction should be addressed during routine visits with the pediatrician, hematologist or gynecologist. Girls who are at risk of being carriers for hemophilia A and B, severe von Willebrand disease, and other severe bleeding disorders should have their bleeding disorder status determined before they become pregnant. During pregnancy, a plan should be established to ensure that both mother and fetus deliver safely. Young women at risk for severe bleeding or at risk of having a severely affected infant should be referred for prenatal care and delivery to a center where, in addition to specialists in high-risk obstetrics, there is a hemophilia treatment center or a hematologist with expertise in hemostasis. Prior to delivery or any invasive procedures, young women at risk for severe bleeding should receive prophylaxis. Since administration of desmopressin may result in hyponatremia, whenever available, virally inactivated or recombinant clotting factor concentrates should be used for replacement as opposed to fresh frozen plasma or cryoprecipitate.

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        • Sadler J.E.
        • Mannucci P.M.
        • Berntorp E.
        • et al.
        Impact, diagnosis and treatment of von Willebrand disease.
        Thromb Haemost. 2000; 84: 160
        • James A.H.
        • Jamison M.G.
        Bleeding events and other complications during pregnancy and childbirth in women with von Willebrand disease.
        J Thromb Haemost. 2007; 5: 1165
        • Biron C.
        • Mahieu B.
        • Rochette A.
        • et al.
        Preoperative screening for von Willebrand disease type 1: low yield and limited ability to predict bleeding.
        J Lab Clin Med. 1999; 134: 605
        • Rodeghiero F.
        • Castaman G.
        • Dini E.
        Epidemiological investigation of the prevalence of von Willebrand's disease.
        Blood. 1987; 69: 454
        • Werner E.J.
        • Broxson E.H.
        • Tucker E.L.
        • et al.
        Prevalence of von Willebrand disease in children: a multiethnic study.
        J Pediatr. 1993; 123: 893
        • James A.H.
        • Lukes A.S.
        • Brancazio L.R.
        • et al.
        Use of a new platelet function analyzer to detect von Willebrand disease in women with menorrhagia.
        Am J Obstet Gynecol. 2004; 191: 449
        • Philipp C.S.
        • Faiz A.
        • Dowling N.
        • et al.
        Age and the prevalence of bleeding disorders in women with menorrhagia.
        Obstet Gynecol. 2005; 105: 61
        • Preventing Teen Pregnancy
        An Update in 2009Adolescent Reproductive Health:About Teen Pregnancy.
        U.S. Department of Health and Human Services Centers for Disease Control and Prevention, Atlanta2009 (vol 2010)
        • Pazol K.
        • Gamble S.B.
        • Parker W.Y.
        • et al.
        Abortion surveillance - United States, 2006.
        MMWR Surveill Summ. 2009; 58: 1
        • Al-Ramahi M.
        • Saleh S.
        Outcome of adolescent pregnancy at a university hospital in Jordan.
        Arch Gynecol Obstet. 2006; 273: 207
      1. Peitsidis P, Datta T, Pafilis I, et al: Bernard Soulier syndrome in pregnancy: a systematic review. Haemophilia.

        • Sherer D.M.
        • Lerner R.
        Glanzmann's thrombasthenia in pregnancy: a case and review of the literature.
        Am J Perinatol. 1999; 16: 297
        • Jallu V.
        • Bianchi F.
        • Kaplan C.
        Fetal-neonatal alloimmune thrombocytopenia and unexpected Glanzmann thrombasthenia carrier: report of two cases.
        Transfusion. 2005; 45: 550
        • Monte S.
        • Lyons G.
        Peripartum management of a patient with Glanzmann's thrombasthenia using Thrombelastograph.
        Br J Anaesth. 2002; 88: 734
        • Evron S.
        • Anteby S.O.
        • Brzezinsky A.
        • et al.
        Congenital afibrinogenemia and recurrent early abortion: a case report.
        Eur J Obstet Gynecol Reprod Biol. 1985; 19: 307
        • Inamoto Y.
        • Terao T.
        First report of case of congenital afibrinogenemia with successful delivery.
        Am J Obstet Gynecol. 1985; 153: 803
        • Goodwin T.M.
        Congenital hypofibrinogenemia in pregnancy.
        Obstet Gynecol Surv. 1989; 44: 157
        • Trehan A.K.
        • Fergusson I.L.
        Congenital afibrinogenaemia and successful pregnancy outcome. Case report.
        Br J Obstet Gynaecol. 1991; 98: 722
        • Lak M.
        • Keihani M.
        • Elahi F.
        • et al.
        Bleeding and thrombosis in 55 patients with inherited afibrinogenaemia.
        Br J Haematol. 1999; 107: 204
        • Kobayashi T.
        • Asahina T.
        • Maehara K.
        • et al.
        Congenital afibrinogenemia with successful delivery.
        Gynecol Obstet Invest. 1996; 42: 66
        • Grech H.
        • Majumdar G.
        • Lawrie A.S.
        • et al.
        Pregnancy in congenital afibrinogenaemia: report of a successful case and review of the literature.
        Br J Haematol. 1991; 78: 571
        • Kobayashi T.
        • Kanayama N.
        • Tokunaga N.
        • et al.
        Prenatal and peripartum management of congenital afibrinogenaemia.
        Br J Haematol. 2000; 109: 364
        • Yamanaka Y.
        • Takeuchi K.
        • Sugimoto M.
        • et al.
        Dysfibrinogenemia during pregnancy treated successfully with fibrinogen.
        Acta Obstet Gynecol Scand. 2003; 82: 972
        • Roque H.
        • Stephenson C.
        • Lee M.J.
        • et al.
        Pregnancy-related thrombosis in a woman with congenital afibrinogenemia: a report of two successful pregnancies.
        Am J Hematol. 2004; 76: 267
        • Rodeghiero F.
        • Castaman G.C.
        • Di Bona E.
        • et al.
        Successful pregnancy in a woman with congenital factor XIII deficiency treated with substitutive therapy. Report of a second case.
        Blut. 1987; 55: 45
        • Kobayashi T.
        • Terao T.
        • Kojima T.
        • et al.
        Congenital factor XIII deficiency with treatment of factor XIII concentrate and normal vaginal delivery.
        Gynecol Obstet Invest. 1990; 29: 235
        • Burrows R.F.
        • Ray J.G.
        • Burrows E.A.
        Bleeding risk and reproductive capacity among patients with factor XIII deficiency: a case presentation and review of the literature.
        Obstet Gynecol Surv. 2000; 55: 103
        • Inbal A.
        • Kenet G.
        Pregnancy and surgical procedures in patients with factor XIII deficiency.
        Biomedical Progress. 2003; 16: 69
        • James A.H.
        More than menorrhagia: a review of the obstetric and gynaecological manifestations of bleeding disorders.
        Haemophilia. 2005; 11: 295
        • Edwards A.
        • Ellwood D.A.
        Ultrasonographic evaluation of the postpartum uterus.
        Ultrasound Obstet Gynecol. 2000; 16: 640
        • Marchant S.
        • Alexander J.
        • Garcia J.
        • et al.
        A survey of women's experiences of vaginal loss from 24 hours to three months after childbirth (the BLiPP study).
        Midwifery. 1999; 15: 72
        • Visness C.M.
        • Kennedy K.I.
        • Ramos R.
        The duration and character of postpartum bleeding among breast-feeding women.
        Obstet Gynecol. 1997; 89: 159
        • Dahlman T.
        • Hellgren M.
        • Blomback M.
        Changes in blood coagulation and fibrinolysis in the normal puerperium.
        Gynecol Obstet Invest. 1985; 20: 37
        • Lee C.Y.
        • Madrazo B.
        • Drukker B.H.
        Ultrasonic evaluation of the postpartum uterus in the management of postpartum bleeding.
        Obstet Gynecol. 1981; 58: 227
        • Hoveyda F.
        • Mackenzie I.Z.
        Secondary postpartum haemorrhage: incidence, morbidity and current management.
        Bjog. 2001; 108: 927
        • Ramsahoye B.
        • Davies S.
        • Dasani H.
        • et al.
        Obstetric management in von Willebrand's disease: a report of 24 pregnancies and a review of the literature.
        Haemophilia. 1995; 1: 140
        • Kadir R.A.
        • Lee C.A.
        • Sabin C.A.
        • et al.
        Pregnancy in women with von Willebrand's disease or factor XI deficiency.
        Br J Obstet Gynaecol. 1998; 105: 314
        • Greer I.A.
        • Lowe G.D.
        • Walker J.J.
        • et al.
        Haemorrhagic problems in obstetrics and gynaecology in patients with congenital coagulopathies.
        Br J Obstet Gynaecol. 1991; 98: 909
        • Kadir R.A.
        • Economides D.L.
        • Braithwaite J.
        • et al.
        The obstetric experience of carriers of haemophilia.
        Br J Obstet Gynaecol. 1997; 104: 803
        • Philipp C.S.
        • Faiz A.
        • Dowling N.F.
        • et al.
        Development of a screening tool for identifying women with menorrhagia for hemostatic evaluation.
        Am J Obstet Gynecol. 2008; 198: 163 e1
        • Kadir R.A.
        Women and inherited bleeding disorders: pregnancy and delivery.
        Semin Hematol. 1999; 36: 28
        • Michaelides K.
        • Tuddenham E.G.
        • Turner C.
        • et al.
        Live birth following the first mutation specific pre-implantation genetic diagnosis for haemophilia A.
        Thromb Haemost. 2006; 95: 373
        • Bolton-Maggs P.H.
        • Perry D.J.
        • Chalmers E.A.
        • et al.
        The rare coagulation disorders–review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation.
        Haemophilia. 2004; 10: 593
        • Lee C.A.
        • Chi C.
        • Pavord S.R.
        • et al.
        The obstetric and gynaecological management of women with inherited bleeding disorders–review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors' Organization.
        Haemophilia. 2006; 12: 301
        • James A.H.
        Guidelines for bleeding disorders in women.
        Thromb Res. 2009; 123: S124
        • Lee C.A.
        • Abdul-Kadir R.
        von Willebrand disease and women's health.
        Semin Hematol. 2005; 42: 42
        • Nichols W.L.
        • Hultin M.B.
        • James A.H.
        • et al.
        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: 171
        • Mahlangu J.N.
        • Gilham A.
        Guideline for the treatment of haemophilia in South Africa.
        S Afr Med J. 2008; 98: 126
        • Keeling D.
        • Tait C.
        • Makris M.
        Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders.
        Haemophilia. 2008;
        • Mannucci P.M.
        How I treat patients with von Willebrand disease.
        Blood. 2001; 97: 1915
        • Ray J.G.
        DDAVP use during pregnancy: an analysis of its safety for mother and child.
        Obstet Gynecol Surv. 1998; 53: 450
        • Oral E.
        • Cagdas A.
        • Gezer A.
        • et al.
        Hematological abnormalities in adolescent menorrhagia.
        Arch Gynecol Obstet. 2002; 266: 72
        • Sanchez-Luceros A.
        • Meschengieser S.S.
        • Turdo K.
        • et al.
        Evaluation of the clinical safety of desmopressin during pregnancy in women with a low plasmatic von Willebrand factor level and bleeding history.
        Thromb Res. 2007; 120: 387
        • Chediak J.R.
        • Alban G.M.
        • Maxey B.
        von Willebrand's disease and pregnancy: management during delivery and outcome of offspring.
        Am J Obstet Gynecol. 1986; 155: 618
        • Pasi K.J.
        • Collins P.W.
        • Keeling D.M.
        • et al.
        Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors' Organization.
        Haemophilia. 2004; 10: 218
        • Kulkarni R.
        • Soucie J.M.
        • Lusher J.
        • et al.
        Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: a report from The Centers for Disease Control and Prevention's (CDC) Universal Data Collection (UDC) project.
        Haemophilia. 2009; 15: 1281
        • Prendiville W.J.
        • Harding J.E.
        • Elbourne D.R.
        • et al.
        The Bristol third stage trial: active versus physiological management of third stage of labour.
        Bmj. 1988; 297: 1295
        • James A.H.
        • Kouides P.A.
        • Abdul-Kadir R.
        • et al.
        Von Willebrand disease and other bleeding disorders in women: consensus on diagnosis and management from an international expert panel.
        Am J Obstet Gynecol. 2009; 201: 12 e1
        • Roque H.
        • Funai E.
        • Lockwood C.J.
        von Willebrand disease and pregnancy.
        J Matern Fetal Med. 2000; 9: 257
        • Kouides P.A.
        Obstetric and gynaecological aspects of von Willebrand disease.
        Best Pract Res Clin Haematol. 2001; 14: 381
        • Claessens E.A.
        • Cowell C.A.
        Acute adolescent menorrhagia.
        Am J Obstet Gynecol. 1981; 139: 277
        • Smith Y.R.
        • Quint E.H.
        • Hertzberg R.B.
        Menorrhagia in adolescents requiring hospitalization.
        J Pediatr Adolesc Gynecol. 1998; 11: 13
        • Bevan J.A.
        • Maloney K.W.
        • Hillery C.A.
        • et al.
        Bleeding disorders: A common cause of menorrhagia in adolescents.
        J Pediatr. 2001; 138: 856
        • Jayasinghe Y.
        • Moore P.
        • Donath S.
        • et al.
        Bleeding disorders in teenagers presenting with menorrhagia.
        Aust N Z J Obstet Gynaecol. 2005; 45: 439
        • Mikhail S.
        • Varadarajan R.
        • Kouides P.
        The prevalence of disorders of haemostasis in adolescents with menorrhagia referred to a haemophilia treatment centre.
        Haemophilia. 2007; 13: 627