Download PDF
Semin Thromb Hemost 2012; 38(07): 720-726
DOI: 10.1055/s-0032-1326787
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Common Management Issues in Pediatric Patients with Mild Bleeding Disorders

Sarah H. O'Brien
1   Center for Innovation in Pediatric Practice, The Research Institute at Nationwide Children's Hospital, Columbus, Ohio
2   Division of Pediatric Hematology and Oncology, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio
› Author Affiliations
Further Information

Publication History

Publication Date:
03 October 2012 (online)

    Permissions and Reprints

Abstract

Type 1 von Willebrand disease and mild platelet function defects are among the most common disorders seen by pediatric hematologists. The management and prevention of bleeding in these patients can be challenging, as there are limited published data to guide clinical practice, and a complete lack of randomized clinical trials. Desmopressin (DDAVP) and antifibrinolytics are the mainstays of treatment in these patients, yet the optimal dosing and timing of these agents to prevent or resolve bleeding, while minimizing adverse side effects, is sometimes unclear. DDAVP-induced hyponatremia is a particularly under-recognized complication in children with bleeding disorders who undergo surgery. Clinicians need to be aware of local measures that are equally important in treating problems such as epistaxis and surgical bleeding. This review will discuss the published literature and provide practical suggestions regarding four common management issues in the care of children and adolescents with mild bleeding disorders: epistaxis, heavy menstrual bleeding, dental extractions, and tonsillectomy.

Keywords

epistaxis - menorrhagia - tonsillectomy - dental extraction - von Willebrand disease
 

  • References

  • 1 National Institutes of Health, National Heart, Lung, and Blood Institute (NHLBI). The diagnosis, evaluation, and management of von Willebrand Disease. Bethesda (MD): National Institute of Health: Department of Health and Human Services; NIH Pub. No. 08–5832. 2007: 112
    Search in Google Scholar
  • 2 Federici AB, Mannucci PM. Management of inherited von Willebrand disease in 2007. Ann Med 2007; 39 (5) 346-358
    CrossrefPubMedSearch in Google Scholar
  • 3 Favaloro EJ, Franchini M, Lippi G. Biological therapies for von Willebrand disease. Expert Opin Biol Ther 2012; 12 (5) 551-564
    CrossrefPubMedSearch in Google Scholar
  • 4 Favaloro EJ. Von Willebrand disease: local diagnosis and management of a globally distributed bleeding disorder. Semin Thromb Hemost 2011; 37 (5) 440-455
    Thieme ConnectPubMedSearch in Google Scholar
  • 5 Mikhail S, Kouides P. von Willebrand disease in the pediatric and adolescent population. J Pediatr Adolesc Gynecol 2010; 23 (6, Suppl) S3-S10
    CrossrefPubMedSearch in Google Scholar
  • 6 Mannucci PM. Treatment of von Willebrand's Disease. N Engl J Med 2004; 351 (7) 683-694
    CrossrefPubMedSearch in Google Scholar
  • 7 Bond L, Bevan D. Myocardial infarction in a patient with hemophilia treated with DDAVP. N Engl J Med 1988; 318 (2) 121
    PubMedSearch in Google Scholar
  • 8 Byrnes JJ, Larcada A, Moake JL. Thrombosis following desmopressin for uremic bleeding. Am J Hematol 1988; 28 (1) 63-65
    CrossrefPubMedSearch in Google Scholar
  • 9 McKay H, Derome F, Haq MA , et al. Bleeding risks associated with inheritance of the Quebec platelet disorder. Blood 2004; 104 (1) 159-165
    CrossrefPubMedSearch in Google Scholar
  • 10 Yang ZG, Chen WP, Wu LD. Effectiveness and safety of tranexamic acid in reducing blood loss in total knee arthroplasty: a meta-analysis. J Bone Joint Surg Am 2012; (e-pub ahead of print). doi: 10.2106/JBJS.K.00873
    PubMedSearch in Google Scholar
  • 11 Adler Ma SC, Brindle W, Burton G , et al. Tranexamic acid is associated with less blood transfusion in off-pump coronary artery bypass graft surgery: a systematic review and meta-analysis. J Cardiothorac Vasc Anesth 2011; 25 (1) 26-35
    CrossrefPubMedSearch in Google Scholar
  • 12 McGarry GW. Nosebleeds in children. Clin Evid (Online). 2011 Jan 13; 2011. pii:0311
    PubMed
  • 13 Messner AH. Management of epistaxis in children. In: Basow, DS, ed. UpToDate, Waltham, MA, 2011. Available at: http://www.uptodate.com/contents/management-of-epistaxis-in-children . Accessed May 9, 2012
    PubMed
  • 14 Bernius M, Perlin D. Pediatric ear, nose, and throat emergencies. Pediatr Clin North Am 2006; 53 (2) 195-214
    CrossrefPubMedSearch in Google Scholar
  • 15 Mathiasen RA, Cruz RM. Prospective, randomized, controlled clinical trial of a novel matrix hemostatic sealant in patients with acute anterior epistaxis. Laryngoscope 2005; 115 (5) 899-902
    CrossrefPubMedSearch in Google Scholar
  • 16 Burton MJ, Dorée CJ. Interventions for recurrent idiopathic epistaxis (nosebleeds) in children. Cochrane Database Syst Rev 2004; (1) CD004461
    PubMedSearch in Google Scholar
  • 17 Loughran S, Spinou E, Clement WA, Cathcart R, Kubba H, Geddes NK. A prospective, single-blind, randomized controlled trial of petroleum jelly/Vaseline for recurrent paediatric epistaxis. Clin Otolaryngol Allied Sci 2004; 29 (3) 266-269
    PubMedSearch in Google Scholar
  • 18 Kubba H, MacAndie C, Botma M , et al. A prospective, single-blind, randomized controlled trial of antiseptic cream for recurrent epistaxis in childhood. Clin Otolaryngol Allied Sci 2001; 26 (6) 465-468
    PubMedSearch in Google Scholar
  • 19 Brown AC, Slocum PC, Putthoff SL, Wallace WE, Foresman BH. Exogenous lipoid pneumonia due to nasal application of petroleum jelly. Chest 1994; 105 (3) 968-969
    CrossrefPubMedSearch in Google Scholar
  • 20 Calder N, Kang S, Fraser L, Kunanandam T, Montgomery J, Kubba H. A double-blind randomized controlled trial of management of recurrent nosebleeds in children. Otolaryngol Head Neck Surg 2009; 140 (5) 670-674
    CrossrefPubMedSearch in Google Scholar
  • 21 Halimeh S. Menorrhagia and bleeding disorders in adolescent females. Hamostaseologie 2012; 32 (1) 45-50
    PubMedSearch in Google Scholar
  • 22 ACOG. Management of Anovulatory Bleeding. Washington, DC: American College of Obstetricians and Gynecologists; 2000
    Search in Google Scholar
  • 23 Amesse LS, Pfaff-Amesse T, Leonardi R, Uddin D, French II JA. Oral contraceptives and DDAVP nasal spray: patterns of use in managing vWD-associated menorrhagia: a single-institution study. J Pediatr Hematol Oncol 2005; 27 (7) 357-363
    CrossrefPubMedSearch in Google Scholar
  • 24 Lethaby AE, Cooke I, Rees M. Progesterone or progestogen-releasing intrauterine systems for heavy menstrual bleeding. Cochrane Database Syst Rev 2005; (4) CD002126
    PubMedSearch in Google Scholar
  • 25 Kingman CE, Kadir RA, Lee CA, Economides DL. The use of levonorgestrel-releasing intrauterine system for treatment of menorrhagia in women with inherited bleeding disorders. BJOG 2004; 111 (12) 1425-1428
    CrossrefPubMedSearch in Google Scholar
  • 26 Chi C, Huq FY, Kadir RA. Levonorgestrel-releasing intrauterine system for the management of heavy menstrual bleeding in women with inherited bleeding disorders: long-term follow-up. Contraception 2011; 83 (3) 242-247
    CrossrefPubMedSearch in Google Scholar
  • 27 Chi C, Pollard D, Tuddenham EG, Kadir RA. Menorrhagia in adolescents with inherited bleeding disorders. J Pediatr Adolesc Gynecol 2010; 23 (4) 215-222
    CrossrefPubMedSearch in Google Scholar
  • 28 Ahuja SP, Hertweck SP. Overview of bleeding disorders in adolescent females with menorrhagia. J Pediatr Adolesc Gynecol 2010; 23 (6, Suppl) S15-S21
    CrossrefPubMedSearch in Google Scholar
  • 29 Kouides PA, Byams VR, Philipp CS , et al. Multisite management study of menorrhagia with abnormal laboratory haemostasis: a prospective crossover study of intranasal desmopressin and oral tranexamic acid. Br J Haematol 2009; 145 (2) 212-220
    CrossrefPubMedSearch in Google Scholar
  • 30 Lukes AS, Moore KA, Muse KN , et al. Tranexamic acid treatment for heavy menstrual bleeding: a randomized controlled trial. Obstet Gynecol 2010; 116 (4) 865-875
    CrossrefPubMedSearch in Google Scholar
  • 31 Ragni MV, Jankowitz RC, Jaworski K, Merricks EP, Kloos MT, Nichols TC. Phase II prospective open-label trial of recombinant interleukin-11 in women with mild von Willebrand disease and refractory menorrhagia. Thromb Haemost 2011; 106 (4) 641-645
    Thieme ConnectPubMedSearch in Google Scholar
  • 32 Lethaby A, Augood C, Duckitt K, Farquhar C. Nonsteriodal anti-inflammatory drugs for heavy menstrual bleeding. Cochrane Database Syst Rev 2007; CD0000400
    PubMedSearch in Google Scholar
  • 33 Federici AB, Sacco R, Stabile F, Carpenedo M, Zingaro E, Mannucci PM. Optimising local therapy during oral surgery in patients with von Willebrand disease: effective results from a retrospective analysis of 63 cases. Haemophilia 2000; 6 (2) 71-77
    CrossrefPubMedSearch in Google Scholar
  • 34 Piot B, Sigaud-Fiks M, Huet P, Fressinaud E, Trossaërt M, Mercier J. Management of dental extractions in patients with bleeding disorders. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002; 93 (3) 247-250
    CrossrefPubMedSearch in Google Scholar
  • 35 Bornert F, Clauss F, Gros CI , et al. Hemostatic management in pediatric patients with type I von Willebrand disease undergoing oral surgery: case report and literature review. J Oral Maxillofac Surg 2011; 69 (8) 2086-2091
    CrossrefPubMedSearch in Google Scholar
  • 36 Cullen KA, Hall MJ, Golosinskiy A. Ambulatory Surgery in the United States, 2006. National Health Statistics reports no. 11, revised. Hyattsville, MD: National Center for Health Statistics; 2009
    Search in Google Scholar
  • 37 Lowe D, van der Meulen J, Cromwell D , et al. Key messages from the National Prospective Tonsillectomy Audit. Laryngoscope 2007; 117 (4) 717-724
    CrossrefPubMedSearch in Google Scholar
  • 38 Krishna P, Lee D. Post-tonsillectomy bleeding: a meta-analysis. Laryngoscope 2001; 111 (8) 1358-1361
    CrossrefPubMedSearch in Google Scholar
  • 39 Dunn AL, Cox Gill J. Adenotonsillectomy in patients with desmopressin responsive mild bleeding disorders: a review of the literature. Haemophilia 2010; 16 (5) 711-716
    CrossrefPubMedSearch in Google Scholar
  • 40 Witmer CM, Elden L, Butler RB, Manno CS, Raffini LJ. Incidence of bleeding complications in pediatric patients with type 1 von Willebrand disease undergoing adenotonsillar procedures. J Pediatr 2009; 155 (1) 68-72
    CrossrefPubMedSearch in Google Scholar
  • 41 Rodriguez KD, Sun GH, Pike F, Mandel EM, Casselbrant ML, Chi DH. Post-tonsillectomy bleeding in children with von Willebrand disease: a single-institution experience. Otolaryngol Head Neck Surg 2010; 142 (5) 715-721
    CrossrefPubMedSearch in Google Scholar
  • 42 Allen GC, Armfield DR, Bontempo FA, Kingsley LA, Goldstein NA, Post JC. Adenotonsillectomy in children with von Willebrand disease. Arch Otolaryngol Head Neck Surg 1999; 125 (5) 547-551
    CrossrefPubMedSearch in Google Scholar
  • 43 Jiménez-Yuste V, Prim MP, De Diego JI , et al. Otolaryngologic surgery in children with von Willebrand disease. Arch Otolaryngol Head Neck Surg 2002; 128 (12) 1365-1368
    CrossrefPubMedSearch in Google Scholar
  • 44 Davidson HC, Stapleton AL, Casselbrant ML, Kitsko DJ. Perioperative incidence and management of hyponatremia in vWD patients undergoing adenotonsillectomy. Laryngoscope 2011; 121 (7) 1399-1403
    CrossrefPubMedSearch in Google Scholar