Outcomes In Adult Hemophilia and Von Willebrand Disease Patients Undergoing Surgical and Invasive Procedures

Adults with hemophilia and von Willebrand disease (VWD) frequently require surgical and invasive procedures. There is variability in current practice of peri-operative management in major surgery and in the management of invasive procedures. The purpose of this study was to evaluate outcomes and management strategies in patients with hemophilia and VWD undergoing surgical and invasive procedures in a comprehensive hemophilia center at a tertiary care hospital.

A retrospective review of electronic medical records was carried out from patients with hemophilia and VWD seen at the Weill Cornell Hemophilia Treatment Center undergoing surgery or an invasive procedure from January 2006 to December 2012. Information on demographics, diagnosis, severity, and presence of inhibitors was also collected.

Procedures and treatment strategies were reviewed including the type of procedure, use of factor bolus/DDAVP, continuous infusion(CI), and antifibrinolytics. Outcomes from these cases were reviewed for acute bleeding (<48 hours after procedure), delayed bleeding (>48 hours after procedure), transfusion of blood products, inhibitor development, and post-procedure thrombosis. Data were compared using Fischer’s exact test.

Our study population consisted of 59 patients (mean age=58, range 21-77). Our hemophilia group included 41 with hemophilia A and 7 with hemophilia B. Our hemophilia patients were severe (n=24), mild-mod (n=12), and 12 had inhibitors. We also identified 5 female carriers of hemophilia A.We identified 6 VWD patients including Type 1 (n=3), 2A (n=1), 3 (n=2). We reviewed 34 major surgeries (26 orthopedic, 8 non-orthopedic) and 91 invasive procedures. We covered all patients an initial dose of either factor VIII/IX or DDAVP. In addition, 55.9% of major procedures were covered by continuous factor infusion (CI), whereas only 9.9% of minor procedures were covered by CI. Conversely, antifibrinolytics were used in 14.7% of major surgeries and 34.1% of minor procedures. Antifibrinolytics were used in all types of minor procedures except those involving the genitourinary tract.

Hematologic outcomes are shown in Table 1. We identified 4 cases of acute bleeding and 10 cases of delayed bleeding. We also identified 5 cases of inhibitor development. We identified one thrombotic episode in an orthopedic surgery case. We further examined several classes of procedures including dental, GI endoscopies, GU procedures, biopsies, and GYN procedures in carriers. One transfusion was needed in the case of a liver biopsy; acute bleeding was noted in 2 biopsies and 1 endoscopy. Delayed bleeding accompanied 28.6% of GU procedures and 16.1% of dental procedures. Inhibitor development was noted after 1 GU procedure and 2 dental procedures.

Our adult hemophilia and VWD population undergoing minor procedures showed similar rates of adverse hematologic outcomes compared to major surgery. This finding highlights the significant risk of even minor procedures in adults with bleeding disorders. Appropriate treatment standards should be designed to take into account patient responses, procedure type, and anticipated outcomes.

No relevant conflicts of interest to declare.