2026 Volume 44 Issue 2 Pages 173-178
Synopsis: Vascular Ehlers-Danlos syndrome (vEDS) is a rare disorder characterized by fragility of blood vessels and organs due to mutations in the type III collagen gene (COL3A1). We report a case of vEDS diagnosed following massive intraoperative bleeding during surgery for endometrial cancer. The patient was a 46-year-old nulligravid woman. During open surgery for endometrial cancer, repeated bleeding occurred due to rupture of peripheral vessels. In particular, significant bleeding was observed during pelvic lymphadenectomy owing to rupture of the left common iliac vein and branches of the right internal iliac vein, which necessitated discontinuation of para-aortic lymphadenectomy and termination of the procedure. The total blood loss was 6,720 mL, requiring massive transfusion. The postoperative diagnosis was stage IIIA, grade 1 endometrioid carcinoma. Intraoperative bleeding tendency suggested an underlying disorder, and a history of recurrent subcutaneous hemorrhage raised suspicion of connective tissue abnormality. Postoperative genetic testing revealed a COL3A1 mutation, confirming the diagnosis of vEDS. As vEDS often lacks specific clinical features, preoperative diagnosis is challenging. When intraoperative tissue fragility is observed, vEDS should be considered in the differential diagnosis, and modification of surgical strategy, including modification of the procedure, is required.
