title: General surgery in patients with a bleeding diathesis: how we do it.
abstract: 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.
authors.affiliation: Department of General Surgery, Central Manchester and Manchester Children's University Hospitals NHS Foundation Trust, and University of Manchester, Manchester Royal Infirmary, Oxford Road, Manchester, M13 9WL, UK. kamal.aryal@jpaget.nhs.uk
authors.forename: Kamal R
authors.initials: KR
authors.lastname: Aryal
authors.forename: D
authors.initials: D
authors.lastname: Wiseman
authors.forename: Ajith K
authors.initials: AK
authors.lastname: Siriwardena
authors.forename: Paula H B
authors.initials: PH
authors.lastname: Bolton-Maggs
authors.forename: Charles R M
authors.initials: CR
authors.lastname: Hay
authors.forename: James
authors.initials: J
authors.lastname: Hill
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001778:Blood Coagulation Disorders / Q000628:therapy; D004198:Disease Susceptibility; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019106:Postoperative Hemorrhage / Q000517:prevention & control*; D012189:Retrospective Studies; D012307:Risk Factors; D013514:Surgical Procedures, Operative; D055815:Young Adult