BACKGROUND: This study was undertaken to assess perioperative management, postoperative complications, and the adequacy of perioperative plasma factor levels in a regional hemophilia center. METHODS: A total of 113 consecutive patients (75 men, 38 women; median age 48 years, range 18-86 years) with bleeding disorders undergoing general surgical and endoscopic procedures (144 procedures: 15 urgent, 129 elective) were reviewed. The episodes were identified from a prospectively collected database at a regional hemophilia center from 1998 to the end of 2008. In all, 46% of the surgical patients had hemophilia A, 38% had von Willebrand disease, 6% had hemophilia B, 5% had factor XI deficiency, and 4% had other disorders. RESULTS: Procedures carried out were endoscopic in 40%, minor in 25%, and intermediate and major in 35%. There were two postoperative deaths, both in patients undergoing urgent major procedures. Postoperative complications occurred after 7.6% (4.0% hemorrhagic, 3.6% nonhemorrhagic) of the procedures. Four of six patients with postoperative hemorrhage required further operative intervention. The median dose of clotting factor for Hemophilia A patients was 2240 U for endoscopic procedures, 7500 U for minor procedures, and 23,500 U for intermediate/major procedures. In hemophiliacs, the mean preoperative plasma factor level attained was 129 IU/dl (SD 16) in patients who developed postoperative hemorrhagic complications and 125 IU/dl (SD 37) in those who did not have bleeding, indicating that in no case was hemorrhage attributable to inadequate factor replacement. CONCLUSIONS: General surgical and endoscopic procedures can be performed with low morbidity and mortality rates when there is appropriate factor replacement and good support from the hemophilia team.
BACKGROUND: This study was undertaken to assess perioperative management, postoperative complications, and the adequacy of perioperative plasma factor levels in a regional hemophilia center. METHODS: A total of 113 consecutive patients (75 men, 38 women; median age 48 years, range 18-86 years) with bleeding disorders undergoing general surgical and endoscopic procedures (144 procedures: 15 urgent, 129 elective) were reviewed. The episodes were identified from a prospectively collected database at a regional hemophilia center from 1998 to the end of 2008. In all, 46% of the surgical patients had hemophilia A, 38% had von Willebrand disease, 6% had hemophilia B, 5% had factor XI deficiency, and 4% had other disorders. RESULTS: Procedures carried out were endoscopic in 40%, minor in 25%, and intermediate and major in 35%. There were two postoperative deaths, both in patients undergoing urgent major procedures. Postoperative complications occurred after 7.6% (4.0% hemorrhagic, 3.6% nonhemorrhagic) of the procedures. Four of six patients with postoperative hemorrhage required further operative intervention. The median dose of clotting factor for Hemophilia Apatients was 2240 U for endoscopic procedures, 7500 U for minor procedures, and 23,500 U for intermediate/major procedures. In hemophiliacs, the mean preoperative plasma factor level attained was 129 IU/dl (SD 16) in patients who developed postoperative hemorrhagic complications and 125 IU/dl (SD 37) in those who did not have bleeding, indicating that in no case was hemorrhage attributable to inadequate factor replacement. CONCLUSIONS: General surgical and endoscopic procedures can be performed with low morbidity and mortality rates when there is appropriate factor replacement and good support from the hemophilia team.
Authors: B Brown; D L Steed; M W Webster; M S Makaroun; J A Spero; F A Bontempo; M V Ragni; J H Lewis Journal: Surgery Date: 1986-02 Impact factor: 3.982
Authors: A Srivastava; M Chandy; G D Sunderaj; V Lee; A J Daniel; D Dennison; S C Nair; V Mathews; G Anderson; A Nair; B V Moses; A Sudarsanam Journal: Haemophilia Date: 1998-11 Impact factor: 4.287
Authors: Sara Orsini; Patrizia Noris; Loredana Bury; Paula G Heller; Cristina Santoro; Rezan A Kadir; Nora C Butta; Emanuela Falcinelli; Ana Rosa Cid; Fabrizio Fabris; Marc Fouassier; Koji Miyazaki; Maria Luisa Lozano; Pamela Zúñiga; Claire Flaujac; Gian Marco Podda; Nuria Bermejo; Remi Favier; Yvonne Henskens; Emmanuel De Maistre; Erica De Candia; Andrew D Mumford; Gul Nihal Ozdemir; Ibrahim Eker; Paquita Nurden; Sophie Bayart; Michele P Lambert; James Bussel; Barbara Zieger; Alberto Tosetto; Federica Melazzini; Ana C Glembotsky; Alessandro Pecci; Marco Cattaneo; Nicole Schlegel; Paolo Gresele Journal: Haematologica Date: 2017-04-06 Impact factor: 9.941
Authors: Milcho J Panovski; Igor V Fildishevski; Ljubomir Lj Ognjenovic; Violeta I Dejanova-Ilijevska Journal: Open Access Maced J Med Sci Date: 2017-05-25