Articles, Issue 3/2026, Брой 3/2026, Брой 3/2026, Articles

Von Willebrand disease in paediatric ENT practice – a case series and practical recommendations

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DOI: 10.66223/pediatria.2026.3.007

I. Stancheva

Department of ENT Diseases, Medical University – Sofia
UMBHAL “Tsaritsa Ioanna – ISUL”, Sofia

Abstract

Von Willebrand disease (VWD) is the most common inherited bleeding disorder that often remains unrecognised in childhood. One of its leading manifestations is recurrent epistaxis.

Aim. To present a series of paediatric cases with initial presentation in ear–nose–throat (ENT) practice and to propose a short focused-history questionnaire.

Material and methods. A retrospective series of three clinical cases: a boy with recurrent epistaxis; a family with two brothers, diagnosed preoperatively and following recurrent epistaxis, respectively; and a 3-year-old girl with severe bleeding after adenoidectomy.

Results. In all three cases the routine coagulation parameters proved insufficient – a focused bleeding and family history was decisive. Conclusion. The history often outperforms routine coagulation screening in selecting patients for specific testing, in line with current international recommendations. We propose a short questionnaire and stress the need for clinician training and patient awareness.

Keywords: von Willebrand disease, epistaxis, children, haemostasis, focused history

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Bibliography

  1. Leebeek FWG, Eikenboom JCJ. Von Willebrand disease. N Engl J Med, 375, 2016, 21, 2067-2080.
  2. Sadler JE, Budde U, Eikenboom JCJ, et al. Update on the pathophysiology and classification of von Willebrand disease. J Thromb Haemost, 4, 2006, 10, 2103-2114.
  3. James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv, 5, 2021, 1, 280-300.
  4. Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv, 5, 2021, 1, 301-325.
  5. Laffan MA, Lester W, O’Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline. Br J Haematol, 167, 2014, 4, 453-465.
  6. Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines. Haemophilia, 14, 2008, 2, 171-232.
  7. Bowman M, Hopman WM, Rapson D, et al. The prevalence of symptomatic von Willebrand disease in primary care practice. J Thromb Haemost, 8, 2010, 1, 213-216.
  8. Sandoval C, Dong S, Visintainer P, et al. Clinical and laboratory features of 178 children with recurrent epistaxis. J Pediatr Hematol Oncol, 24, 2002, 1, 47-49.
  9. Biss TT, Blanchette VS, Clark DS, et al. Quantitation of bleeding symptoms in children with von Willebrand disease: use of a standardized pediatric bleeding questionnaire. J Thromb Haemost, 8, 2010, 5, 950-956.
  10. Bowman M, Riddel J, Rand ML, et al. Evaluation of the diagnostic utility for von Willebrand disease of a pediatric bleeding questionnaire. J Thromb Haemost, 7, 2009, 8, 1418-1421.
  11. Rodeghiero F, Tosetto A, Abshire T, et al. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost, 8, 2010, 9, 2063-2065.
  12. National Institute for Health and Care Excellence. Routine preoperative tests for elective surgery (NG45). London, NICE, 2016.
  13. Alzahrani A, Othman N, Bin-Ali T, et al. Routine preoperative coagulation tests in children undergoing elective surgery or invasive procedures: are they still necessary? Clin Med Insights Blood Disord, 12, 2019, 1179545X18821158.
  14. Scheckenbach K, Bier H, Hoffmann TK, et al. [[Risk of hemorrhage after adenoidectomy and tonsillectomy. Value of the preoperative determination of partial thromboplastin time, prothrombin time and platelet count]. HNO, 56, 2008, 3, 312-320.
  15. Thiele T, Kaftan H, Hosemann W, Greinacher A. Hemostatic management of patients undergoing ear-nose-throat surgery. GMS Curr Top Otorhinolaryngol Head Neck Surg, 14, 2015, Doc07.
  16. Mitchell RB, Archer SM, Ishman SL, et al. Clinical practice guideline: tonsillectomy in children (update). Otolaryngol Head Neck Surg, 160, 2019, 1_suppl, S1-S42.
  17. Zwack GC, Derkay CS. The utility of preoperative hemostatic assessment in adenotonsillectomy. Int J Pediatr Otorhinolaryngol, 39, 1997, 1, 67-76.
  18. National Institute for Health and Care Excellence. Clinical evidence review of vonicog alfa for the treatment and prevention of bleeding in adults with von Willebrand disease (URN1709/ID018). London, NICE, 2020.
  19. Mannucci PM. Treatment of von Willebrand’s disease. N Engl J Med, 351, 2004, 7, 683-694.
  20. Banchev A, Batorova A, Faganel Kotnik B, et al. A cross-national survey of people living with hemophilia: impact on daily living and patient education in Central Europe. Patient Prefer Adherence, 15, 2021, 871-883.

Address for correspondence:

Iglika Stancheva

Department of Otorhinolaryngology, Faculty of Medicine, Medical University of Sofia,
University Hospital “Tsaritsa Yoanna – ISUL”, Sofia

8, “Bialo more”, Str.

e-mail: iglika_stancheva@yahoo.com