Colon cancer surgery in von Willebrand disease type 3 setting triggering vascular abnormalities on bowel anastomosis

BACKGROUND: von Willebrand disease (VWD) is associated with vascular malformations in the gastrointestinal tract. This complication, more frequent in VWD types 2A and 3, may be due to abnormal angiogenesis, but the precise mechanism is still unclear. Angiogenesis and inflammation are closely linked and can potentiate each other. KEY CLINICAL QUESTION: Can colon inflammation in the setting of cancer surgery potentiate angiogenesis in the VWD setting? CLINICAL APPROACH: A woman with VWD type 3 underwent partial colectomy twice for an adenocarcinoma. After managing the first surgery with a plasma-derived von Willebrand factor (VWF) concentrate (Wilfactin; LFB), refractory gastrointestinal bleeding occurred from neovessels on bowel anastomosis. After a multidisciplinary discussion, a second surgery was undertaken with a recombinant VWF concentrate (Veyvondi; Takeda Pharmaceuticals). Pathologic neovessels were again observed on the new anastomosis. CONCLUSION: Colectomy was complicated twice by pathologic neovessels on bowel anastomosis in 2 distinct procedures managed either with plasma-derived VWF or with recombinant VWF.