BACKGROUND: Hemophilia and von Willebrand disease (VWD) are the most common inherited bleeding disorders, and hemostatic management surrounding surgical procedures is complex. Hemophilia Treatment Centers (HTCs) devote significant time and resources to formulating perioperative hemostatic plans to allow these patients to safely undergo necessary procedures. Yet, scant data exists in the literature in the era of routine recombinant and plasma-derived factor concentrate use on outcomes of persons with bleeding disorders undergoing major surgeries.

METHODS: We performed a retrospective chart review of surgical outcomes in patients with bleeding disorders at the Cardeza Hemophilia and Thrombosis Center, an HTC at a large academic medical center in Philadelphia, PA. The study included 50 patients, among whom a total of 63 surgeries occurred between 2017-2019. Patients carried a diagnosis of Hemophilia A or B, VWD, or other congenital coagulation factor deficiency. We included planned surgeries requiring general anesthesia for >30 minutes, as well as orthopedic and neurologic surgeries; only procedures occurring at our hospital were included. The primary outcome was postoperative bleeding, assessed according to the ISTH-SSC's 2010 definition. Secondary outcomes included use of unplanned postoperative hemostatic therapy, length of stay, and 30-day readmission rate.

To compare outcomes in our population to that of a non-bleeding disorder population, the American College of Surgeons National Surgery Quality Improvement Program (NSQIP) database was queried for 2018 (N=251,682). Only patients with a primary surgical CPT code shared with our study population were included, and those with a surgery occurring beyond Day 0 of admission or with a known bleeding disorder were excluded. Not all procedures undergone by our patients are captured by NSQIP, so our comparison study population included 40 procedures among 35 patients.

RESULTS: Characteristics of the study population are summarized in Table 1. Among 50 eligible patients in our registry, 21 (42%) were female and mean age at the time of surgery was 50.9 years. The most common diagnosis was VWD (64%), with Type 1 (42%) the most frequent subtype. Most patients (44%) had a mild bleeding disorder. Three (6%) had a severe phenotype, including 1 patient with an inhibitor, and 2 (4%) used chronic outpatient prophylaxis.

Outcomes in the 63 surgeries are summarized in Table 2. The most common surgical procedure category was orthopedic (21, 33.3%), including 12 arthroplasties. 62 patients (98.4%) had a preoperative hemostatic plan documented in the chart. 58 (92.1%) patients received preoperative hemostatic therapy, and 34 (54.0%) had planned postoperative therapy. Postoperatively, 59 patients (93.7%) had no documented bleeding complications. One (1.6%) had clinical bleeding not meeting ISTH major bleeding criteria and 3 (4.8%) had major bleeding, including 2 patients with unexpected surgical site bleeding and Hgb drop ≥2 g/dL and 1 who had a Hgb drop and required 5 units of pRBCs. Seven (11.1%) patients required unplanned postoperative hemostatic therapy. The mean length of stay was 1.65 days, and only 4 patients (6.3%) re-presented to the hospital within 30 days related to surgery - 2 for bleeding complications - including 3 ED visits and 1 inpatient admission.

To understand how this rate of complications compares to the general population at academic centers, we compared study patients to non-bleeding disorder patients in the 2018 NSQIP database. Characteristics of each group are shown in Table 3. NSQIP defines bleeding only as postoperative transfusion, however all bleeding events in the study patients in this comparison included transfusion. Postoperative bleeding rates were low in both groups (3.0% in study patients vs 1.2% in NSQIP controls, P=.082). There was no difference in unplanned 30-day readmission rates related to surgery. Mean length of stay was longer in study patients (2.25 vs 1.90 days, P=.003).

DISCUSSION: This study at a single HTC suggests that, with comprehensive care and preoperative planning, patients with bleeding disorders can safely undergo major surgical procedures with risks of bleeding complications similar to the general population. We observed few bleeding events or readmissions, and length of stay remained relatively short despite most patients being planned for postoperative hemostatic therapy.


Drelich:Octapharma: Honoraria; Bayer: Honoraria; CSL Behring: Honoraria.

Author notes


Asterisk with author names denotes non-ASH members.