title: Outcomes in Patients With Hemophilia and von Willebrand Disease Undergoing Invasive or Surgical Procedures.
abstract: Adults with hemophilia A (HA), hemophilia B (HB), and von Willebrand disease (VWD) frequently require surgery and invasive procedures. However, there is variability in perioperative management guidelines. We describe our periprocedural outcomes in this setting. A retrospective chart review from January 2006 to December 2012 of patients with HA, HB, and VWD undergoing surgery or invasive procedures was conducted. Type of procedures, management including the use of continuous factor infusion, and administration of antifibrinolytics were reviewed. Adverse outcomes were defined as acute bleeding (<48 hours), delayed bleeding (≥48 hours), transfusion, inhibitor development, and thrombosis. We identified 59 patients with HA and HB. In all, 24 patients had severe hemophilia and 12 had mild/moderate hemophilia. Twelve patients had inhibitors. There were also 5 female carriers of HA and 6 patients with VWD. There were 34 major surgeries (26 orthopedic, 8 nonorthopedic) and 129 minor surgeries. Continuous infusion was used in 55.9% of major surgeries versus 8.5% of minor surgeries. Antifibrinolytics were administered in 14.7% of major surgeries versus 23.2% of minor surgeries. In all, 4 patients developed acute bleeding and 10 patients developed delayed bleeding. Delayed bleeding occurred in 28.6% of genitourinary procedures and in 16.1% of dental procedures. Five patients acquired an inhibitor and 2 had thrombosis. In conclusion, patients with HA, HB, or VWD had similar rates of adverse outcomes when undergoing minor surgeries or major surgeries. This finding underscores the importance of an interdisciplinary management and procedure-specific guidelines for patients with hemophilia and VWD prior to even minor invasive procedures.
authors.affiliation: 1 Division of Hematology/Oncology, Weill Cornell Medicine, New York, NY, USA.|2 Weill Cornell Comprehensive Hemophilia Treatment Center, New York, NY, USA.
authors.forename: John
authors.initials: J
authors.lastname: Chapin
authors.affiliation: 1 Division of Hematology/Oncology, Weill Cornell Medicine, New York, NY, USA.
authors.forename: Jaqueline
authors.initials: J
authors.lastname: Bamme
authors.affiliation: 2 Weill Cornell Comprehensive Hemophilia Treatment Center, New York, NY, USA.
authors.forename: Fraustina
authors.initials: F
authors.lastname: Hsu
authors.affiliation: 3 Department of Healthcare Policy and Research, Weill Cornell Medicine, New York, NY, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Christos
authors.affiliation: 1 Division of Hematology/Oncology, Weill Cornell Medicine, New York, NY, USA.|2 Weill Cornell Comprehensive Hemophilia Treatment Center, New York, NY, USA.
authors.forename: Maria
authors.initials: M
authors.lastname: DeSancho
mesh_terms: D000328:Adult; D000368:Aged; D019468:Disease Management; D005260:Female; D006467:Hemophilia A / Q000150:complications / Q000601:surgery; D002836:Hemophilia B / Q000150:complications / Q000601:surgery; D006470:Hemorrhage / Q000209:etiology; D006474:Hemorrhagic Disorders / Q000150:complications* / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D059035:Perioperative Period; D017410:Practice Guidelines as Topic / Q000592:standards; D012189:Retrospective Studies; D013514:Surgical Procedures, Operative / Q000009:adverse effects*; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000150:complications / Q000601:surgery
keywords: complications; hemophilia; invasive procedures; surgery; von Willebrand disease