Factor XIII (FXIII) deficiency is an extremely rare inherited coagulopathy that poses significant perioperative risks of delayed bleeding despite normal routine coagulation tests. Due to the routine normality of the standard coagulation screening tests in the affected patients, the diagnosis needs a high index of clinical suspicion. The implications for the perioperative environment are especially critical in neurosurgical procedures, in which uncontrolled hemorrhage can lead to either stroke, irreversible neurological damage, or death. We discuss the case of a 48-year-old woman with FXIII deficiency and Moyamoya disease who has undergone left-sided encephaloduroarteriosynangiosis (EDAS) surgery after receiving 40 units/kg of plasma-derived FXIII concentrate (Corifact®). Normotension and normocapnia were ensured intraoperatively, and estimated blood loss was 300 milliliters. The hemoglobin levels were stable. The patient was discharged on day 3 after the surgery without any hemorrhagic incidents. We also surveyed the literature on FXIII deficiency in the perioperative environment that has been published so far, especially on diagnostic methods, replacement, and the findings in the neurosurgical patients. Given the limited literature on FXIII deficiency in the context of EDAS and Moyamoya disease, this report contributes valuable clinical insight and supports the need for individualized, evidence-based perioperative strategies.
Chauhan et al. (Fri,) studied this question.