Obstetric and peri-operative management of patients with Factor XI deficiency: a retrospective observational study

Factor XI (FXI) deficiency is an autosomally inherited milder bleeding disorder which may predispose to a potential risk of life-threatening bleeding during childbirth or surgery. Unfortunately, data regarding obstetric and perioperative management of this condition are scarce, with limited cases reviewed in the last decade. Therefore, the present study aimed to expand this database and identify factors associated with increased bleeding risk. We performed a retrospective chart review of patients with FXI deficiency who underwent childbirth or other surgical procedures between August 2011 to April 2021 within a single academic health system and identified 198 patients who underwent 252 procedures, including 143 vaginal deliveries, 63 cesarean (C-sections) and 46 other surgical procedures. Thirty three of 252 procedures resulted in bleeding complications. On multivariable logistic regression analysis, personal history of bleeding was the strongest predictor of perioperative or obstetrical bleeding (OR = 5.92, p=0.001). Higher FXI levels correlated with lower odds of bleeding (OR 0.72 with every 10 U/dl increase in FXI level, p=0.05). On ROC analysis, FXI level of >40 U/dl predicted lower bleeding risk with reasonable specificity (75%) but lacked sensitivity (47%). Family history of bleeding, ethnicity, genotype, pre- procedural PTT and platelet levels were not associated with bleeding risk. There were no cases of epidural or spinal hematomas associated with neuraxial anesthesia. FXI levels remain stable during pregnancy and repeat measurements may not be necessary.