Peri-operative management of children with von Willebrand Factor Deficiency
Nadia THEOLOGIE-LYGIDAKIS, Andreas KOUROUMALIS, Helen PLATOKOUKI, Chrysanthi MATSIOULA, Ioannis IATROU
University Department of Oral and Maxillofacial Surgery, at “P. & A. Kyriakou” Children’s Hospital (Head: Professor I. Iatrou), Dental School, National and Kapodistrian University of Athens, Haemostasis Unit and Hemophilia Center at “Aghia Sophia” Children’s Hospital, (Head: Dr H. Platokouki), Athens, Greece
Hellenic Archives of Oral & Maxillofacial Surgery (2015) 1, 3-10
SUMMARY: Von Willebrand’s disease is an inherited bleeding disorder caused by a deficiency of von Willebrand Factor. In children population, a tooth extraction or a maxillofacial operation may first reveal the deficiency. In order to perform an operation, temporal decrease of the bleeding tendency may be achieved with desmopressin, a synthetic analogue of antidiuretic hormone.
The aim of this study is to present the management of children with von Willebrand Factor deficiency who underwent surgical interventions for maxillofacial problems. Results: The peri-operative treatment of young patients with desmopressin, in order to undergo maxillofacial operations, was successful. Local hemostatic measurements controlled bleeding in cases of previously undiagnosed Von Willebrand’s disease.
Conclusions: Initial diagnosis of the deficiency may be established following unexpected bleeding in oral surgery cases, or following preoperative blood tests in case of maxillofacial operations.
KEY WORDS: von Willebrand Factor Deficiency, Oral and Maxillofacial Surgery, children, desmopressin
REFERENCES
Ben-Ami T, Revel-Vilk S: The use of DDAVP in children with bleeding disorders. Pediatr Blood Cancer 60:s41-43, 2013
Castaman G, Coppola A, Zanon E. et al.: Efficacy and safety during for- mulation switch of a pasteurized VWF/FVIII concentrate: results from an Italian prospective observational study in patients with von Willebrand disease. Haemophilia. 19(1):82-8, 2013
Castaman G, Montgomery RR, Meschengieser SS et al.: Von Wille- brand’s disease diagnosis and laboratory issues. Haemophilia 16(5):67-73, 2010
Castaman G.: Treatment of von Willebrand disease with VFIII/VWF concentrates. Blood Transfuse 9(2):s9-s13, 2011
De Meyer SF, Deckmyn H, Vanhoorelbeke K.: von Willebrand factor to the rescue. Blood 113(21):5049-57, 2009
Favaloro JE: Rethinking the diagnoses of von Willebrand disease.
Thromb Res 127(2):s17-s21, 2011
Federici AB, Bucciarelli P, Castaman G. et al.: Management of inherited von Willebrand disease in Italy: results from the retrospective study on 1234 patients. Semin Thromb Hemost 37(5):511-521, 2011
Federici AB, Castaman G, Franchini M. et al.: Clinical use of Haemate® P in inherited von Willebrand’s disease: a cohort study on 100 Italian patients. Haematologica 92(07):944-951, 2007
Federici AB.: Prophylaxis of bleeding episodes in patients with von Willebrand’s disease. Blood Transfus 6(2):s26-s32, 2008
Franchini M, Lippi G.: The management of patients with congenital von Willebrand disease during surgery or other invasive procedures: focus on antihemophilic factor/von Willebrand factor complex. Biologics 1(3):285-289, 2007
Franchini M, Rossetti G, Tagliaferri A. et al.: Efficacy and safety of factor VIII/von Willebrand's factor concentrate (Haemate-P) in prevent- ing bleeding during surgery or invasive procedures in patients with von Willebrand disease. Haematologica 88(11):1279-83, 2003
Franchini M: Surgical prophylaxis in von Willebrand’s disease: a difficult balance to manage. Blood Transfus 6(2):s33-s38, 2008
James AH: More than menorrhagia: a review of the obstetric and gy- naecological manifestations of von Willebrand disease. Thromb Res 120(1):s17-20, 2007
Laffan MA, Lester W, O’Donnell JS et al.: The diagnosis and manage- ment of von Willebrand disease: a United Kingdom Haemophillia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br. J. Haematol 167:453-465, 2014
Luo GP, Ni B, Yang X et al.: Von Willebrand Factor: More Than a Reg- ulator of Hemostasis and Thrombosis. Acta Haematol 128:158- 169, 2012
Mannucci PM, Federici AB, James AH et al: Von Willebrand disease in the 21st century:current approaches and new challenges. Haemo- philia 15:1154-1158, 2009
Mazzeffi AM, Stone EM: Perioperative management of von Willebrand disease: a review for the anesthesiologist. J Clin Anesth 23:418- 426, 2011
Nichols WL, Hultin MB, James AH 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 14(2):171-232, 2008
Pasi KJ, Collins PW, Keeling DM et al.: Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors’ Organization. Haemophilia 10:218-231, 2004
Piot B, Sigaud-Fiks M, Huet P, et al.: Management of dental extractions in patients with bleeding disorders. Oral Surg Oral Med Oral Pathol 93(3):247-250, 2002
Rodeghiero F.: Von Willebrand disease: pathogenesis and management Thromb Res 131(1):s47-s50, 2013
Sadler JE, Budde U, Eikenboom JC et al.: Update on the pathophysiol- ogy and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost 4(10):2103-14, 2006
Sakurai Y, Shima M, Imai Y et al.: Successful use of recombinant factor VIII devoid of von Willebrand factor during multiple teeth extrac- tions in a patient with type 3 von Willebrand disease. Blood Co- agul Fibrinolysis 17(2):151-154, 2006
Sharma R, Stein D.: Hyponatremia after desmopressin (DDAVP) use in pediatric patients with bleeding disorders undergoing surgeries. J Pediatr Hematol Oncol 36(6):e371-e375, 2014
University Department of Oral and Maxillofacial Surgery, at “P. & A. Kyriakou” Children’s Hospital (Head: Professor I. Iatrou), Dental School, National and Kapodistrian University of Athens, Haemostasis Unit and Hemophilia Center at “Aghia Sophia” Children’s Hospital, (Head: Dr H. Platokouki), Athens, Greece
Hellenic Archives of Oral & Maxillofacial Surgery (2015) 1, 3-10
SUMMARY: Von Willebrand’s disease is an inherited bleeding disorder caused by a deficiency of von Willebrand Factor. In children population, a tooth extraction or a maxillofacial operation may first reveal the deficiency. In order to perform an operation, temporal decrease of the bleeding tendency may be achieved with desmopressin, a synthetic analogue of antidiuretic hormone.
The aim of this study is to present the management of children with von Willebrand Factor deficiency who underwent surgical interventions for maxillofacial problems. Results: The peri-operative treatment of young patients with desmopressin, in order to undergo maxillofacial operations, was successful. Local hemostatic measurements controlled bleeding in cases of previously undiagnosed Von Willebrand’s disease.
Conclusions: Initial diagnosis of the deficiency may be established following unexpected bleeding in oral surgery cases, or following preoperative blood tests in case of maxillofacial operations.
KEY WORDS: von Willebrand Factor Deficiency, Oral and Maxillofacial Surgery, children, desmopressin
REFERENCES
Ben-Ami T, Revel-Vilk S: The use of DDAVP in children with bleeding disorders. Pediatr Blood Cancer 60:s41-43, 2013
Castaman G, Coppola A, Zanon E. et al.: Efficacy and safety during for- mulation switch of a pasteurized VWF/FVIII concentrate: results from an Italian prospective observational study in patients with von Willebrand disease. Haemophilia. 19(1):82-8, 2013
Castaman G, Montgomery RR, Meschengieser SS et al.: Von Wille- brand’s disease diagnosis and laboratory issues. Haemophilia 16(5):67-73, 2010
Castaman G.: Treatment of von Willebrand disease with VFIII/VWF concentrates. Blood Transfuse 9(2):s9-s13, 2011
De Meyer SF, Deckmyn H, Vanhoorelbeke K.: von Willebrand factor to the rescue. Blood 113(21):5049-57, 2009
Favaloro JE: Rethinking the diagnoses of von Willebrand disease.
Thromb Res 127(2):s17-s21, 2011
Federici AB, Bucciarelli P, Castaman G. et al.: Management of inherited von Willebrand disease in Italy: results from the retrospective study on 1234 patients. Semin Thromb Hemost 37(5):511-521, 2011
Federici AB, Castaman G, Franchini M. et al.: Clinical use of Haemate® P in inherited von Willebrand’s disease: a cohort study on 100 Italian patients. Haematologica 92(07):944-951, 2007
Federici AB.: Prophylaxis of bleeding episodes in patients with von Willebrand’s disease. Blood Transfus 6(2):s26-s32, 2008
Franchini M, Lippi G.: The management of patients with congenital von Willebrand disease during surgery or other invasive procedures: focus on antihemophilic factor/von Willebrand factor complex. Biologics 1(3):285-289, 2007
Franchini M, Rossetti G, Tagliaferri A. et al.: Efficacy and safety of factor VIII/von Willebrand's factor concentrate (Haemate-P) in prevent- ing bleeding during surgery or invasive procedures in patients with von Willebrand disease. Haematologica 88(11):1279-83, 2003
Franchini M: Surgical prophylaxis in von Willebrand’s disease: a difficult balance to manage. Blood Transfus 6(2):s33-s38, 2008
James AH: More than menorrhagia: a review of the obstetric and gy- naecological manifestations of von Willebrand disease. Thromb Res 120(1):s17-20, 2007
Laffan MA, Lester W, O’Donnell JS et al.: The diagnosis and manage- ment of von Willebrand disease: a United Kingdom Haemophillia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br. J. Haematol 167:453-465, 2014
Luo GP, Ni B, Yang X et al.: Von Willebrand Factor: More Than a Reg- ulator of Hemostasis and Thrombosis. Acta Haematol 128:158- 169, 2012
Mannucci PM, Federici AB, James AH et al: Von Willebrand disease in the 21st century:current approaches and new challenges. Haemo- philia 15:1154-1158, 2009
Mazzeffi AM, Stone EM: Perioperative management of von Willebrand disease: a review for the anesthesiologist. J Clin Anesth 23:418- 426, 2011
Nichols WL, Hultin MB, James AH 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 14(2):171-232, 2008
Pasi KJ, Collins PW, Keeling DM et al.: Management of von Willebrand disease: a guideline from the UK Haemophilia Centre Doctors’ Organization. Haemophilia 10:218-231, 2004
Piot B, Sigaud-Fiks M, Huet P, et al.: Management of dental extractions in patients with bleeding disorders. Oral Surg Oral Med Oral Pathol 93(3):247-250, 2002
Rodeghiero F.: Von Willebrand disease: pathogenesis and management Thromb Res 131(1):s47-s50, 2013
Sadler JE, Budde U, Eikenboom JC et al.: Update on the pathophysiol- ogy and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost 4(10):2103-14, 2006
Sakurai Y, Shima M, Imai Y et al.: Successful use of recombinant factor VIII devoid of von Willebrand factor during multiple teeth extrac- tions in a patient with type 3 von Willebrand disease. Blood Co- agul Fibrinolysis 17(2):151-154, 2006
Sharma R, Stein D.: Hyponatremia after desmopressin (DDAVP) use in pediatric patients with bleeding disorders undergoing surgeries. J Pediatr Hematol Oncol 36(6):e371-e375, 2014
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