Obstetric and perioperative management of patients with factor XI deficiency: a retrospective observational study

Factor XI (FXI) deficiency is an autosomal inherited, milder bleeding disorder that 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 and April 2021 within a single academic health system and identified 198 patients who underwent 252 procedures, including 143 vaginal deliveries, 63 cesarean deliveries, and 46 other surgical procedures. Thirty-three of the 252 procedures resulted in bleeding complications. On multivariable logistic regression analysis, personal history of bleeding was the strongest predictor of perioperative or obstetric bleeding (odds ratio [OR], 5.92; P = .001). Higher FXI levels were correlated with lower odds of bleeding (OR, 0.72 with every 10 U/dL increase in FXI level; P = .05). On receiver operative characteristic analysis, FXI level of >40 U/dL predicted a lower bleeding risk with reasonable specificity (75%) but lacked sensitivity (47%). A family history of bleeding, ethnicity, genotype, preprocedural partial thromboplastin time, and platelet levels were not associated with bleeding risk. There were no cases of epidural or spinal hematoma associated with neuraxial anesthesia. FXI levels remain stable during pregnancy and repeated measurements may not be necessary.