Low endoscopy bleeding risk in patients with congenital bleeding disorders

Abstract

Introduction Haemophilia A and haemophilia B, von Willebrand disease (VWD), factor VII deficiency and factor XI deficiency are congenital bleeding disorders predisposing to bleeding during invasive procedures. The ageing population of people with congenital bleeding disorders will likely increasingly require gastrointestinal endoscopy. The bleeding risk postgastrointestinal endoscopy and optimal prophylactic treatment regimens are not well described. Methods We performed a retrospective chart review at the McGill University Health Centre. Adult patients with haemophilia A or B, VWD, FVII deficiency and FXI deficiency who underwent gastrointestinal endoscopic procedures were included. Bleeding prophylaxis included combinations of plasma‐derived factor (VWD) or recombinant factor (haemophilia A and haemophilia B), desmopressin and/or tranexamic acid. Our primary outcome was the 72‐hour postendoscopy bleeding rate. Results One hundred and four endoscopies were performed in 48 patients. Haemophilia A (45.3% of endoscopies) was the most common bleeding disorder, followed by VWD (38.5%), FXI deficiency (8.7%), haemophilia B (4.8%) and FVII deficiency (2.9%). All patients were reviewed by the Haemophilia Treatment Center with peri‐procedure treatment protocols put in place as required. The overall 72‐hour bleeding rate was 0.96%, confidence interval (CI) 95% (0.17%‐5.25%). The colonoscopic postpolypectomy bleeding rate was 1/21 (4.8%, CI 95% (0.9%‐22.7%)) in comparison with the general population rate of 0.3%‐10% for high‐risk endoscopy (including colonoscopic polypectomy). Conclusion To the best of our knowledge, this is the largest study describing patients with inherited bleeding disorders undergoing gastrointestinal endoscopy. The bleeding risk is not significantly higher to the general population when haemostatically managed by a team experienced in bleeding disorders.

Hemostatic prophylaxis and colonoscopy outcomes for patients with bleeding disorders: A retrospective cohort study and review of the literature

“…To our knowledge, our study is the largest series to date to describe outcomes of periprocedural management strategies for colonoscopy in PWBD and may help to guide periprocedural HP recommendations and/or future studies. During 75 of 141 (53%) colonoscopy procedures in our study, no intervention was performed, findings that are similar to those of others, which are described in detail in the next paragraph . We therefore speculate that, based on the ATHN report, approximately half of the 12 000 expected colonoscopies over the next decade may not result in an intervention.…”

“…During 75 of 141 (53%) colonoscopy procedures in our study, no intervention was performed, findings that are similar to those of others, which are described in detail in the next paragraph. [8][9][10] We therefore speculate that, based on the ATHN report, approximately half of the 12 000 expected colonoscopies over the next decade may not result in an intervention. Bleeding risk varies by intervention type.…”

“…[1/141]) was similar to that of the general population (0.1%-0.6%) 13 and lower than that reported previously. [8][9][10] Furthermore, categorization (major or minor bleeding) and timing of onset of bleeding complications (in relation to intervention) are unique features of our study. Although early onset (procedural) or immediate postprocedural bleeding might be expected in the PWBD population, our study did not support this hypothesis.…”

“…One was an abstract with insufficient details to compare to our study, 17 and three were full-length publications that we included here. [8][9][10] In a prospective study by Davis et al, 8 28 PWBD (severe and mild haemophilia, 9 and 12, respectively; and moderate VWD, 5) underwent 32 endoscopic procedures, 20 of which were colonoscopy procedures; 10 of the 20 colonoscopies (50%) did not require an intervention. All patients received TXA (1 g orally every 8 hours), starting the night before the procedure and continuing for 10 days for a standard risk intervention (eg, mucosal biopsy or polypectomy < 10 mm).…”

“…One was an abstract with insufficient details to compare to our study, 17 and three were full-length publications that we included here. [8][9][10] In a prospective Baseline factor levels provided for VWD patients are ristocetin cofactor activity levels. Others are the coagulation factor activity levels appropriate for the underlying bleeding disorder.…”