Factor XI deficiency and orthognathic surgery: a case report on anesthesia management

Abstract

Factor XI deficiency (Hemophilia C) is a very rare autosomal recessive bleeding disorder. Patients with factor XI deficiency do not typically show any spontaneous bleeding or specific symptoms. Sometimes those who have this disorder are identified during special situations such as trauma or surgery. Orthognathic surgery is particularly associated with a high bleeding risk. Therefore, great care must be taken when treating patients with bleeding disorders such as factor XI deficiency. There are a few reports that address the management of patients with bleeding disorders during orthognathic surgery. The current report describes a patient with factor XI deficiency who underwent Le Fort I osteotomy together with bilateral sagittal split osteotomy. The patient's condition was assessed using both rotation thromboelastometry (ROTEM™) and noninvasive measurements of total hemoglobin (SpHb) using Masimo Radical 7 (Masimo Co. CA, USA).

First‐in‐human study of milvexian, an oral, direct, small molecule factor XIa inhibitor

“…In patients with FXI deficiency, spontaneous bleeding is rare and bleeding risk is poorly correlated with plasma levels of FXI 10 . In most cases, patients with FXI deficiency do not exhibit symptoms or require treatment, and the only clinical manifestation is an increased tendency for mild bleeding after serious injury or surgery 11–13 . Importantly, clinical, preclinical, and epidemiologic studies have shown that FXI deficiency is associated with reduced risk of adverse cardiovascular events and venous thromboembolism 14–18 .…”

“… 10 In most cases, patients with FXI deficiency do not exhibit symptoms or require treatment, and the only clinical manifestation is an increased tendency for mild bleeding after serious injury or surgery. 11 , 12 , 13 Importantly, clinical, preclinical, and epidemiologic studies have shown that FXI deficiency is associated with reduced risk of adverse cardiovascular events and venous thromboembolism. 14 , 15 , 16 , 17 , 18 In contrast, a large, population‐based, case‐control study (the Leiden Thrombophilia Study) found that patients who had suffered a deep vein thrombosis had significantly higher FXI levels compared with healthy controls, with the risk of thromboembolism doubled for individuals with FXI levels above the 90th population percentile compared with those who had lower values.…”

Single-Dose Pharmacokinetics of Milvexian in Participants with Mild or Moderate Hepatic Impairment Compared with Healthy Participants

“…Participants remained at the clinical facility, and serial pharmacokinetic samples were collected for up to 72 h after study drug administration for healthy participants and for up to 96 h for participants with hepatic impairment. spontaneous bleeding events [8][9][10][11][12][13]. In contrast, individuals with elevated plasma FXI levels have been shown to be at increased risk for venous thrombosis [14].…”

Single-Dose Pharmacokinetics of Milvexian in Participants with Normal Renal Function and Participants with Moderate or Severe Renal Impairment

“…Based on findings from clinical and preclinical models, hemostasis is not solely dependent on the FXI pathway, and FXIa inhibitors have the potential to reduce thrombus formation [ 17 – 20 ]. Notably, spontaneous bleeding is rare in individuals with congenital FXI deficiencies and, for these individuals, mild bleeding after a serious injury or surgery is the only clinical manifestation of FXI deficiency [ 21 – 23 ]. Additionally, results from in vivo and clinical studies have shown that there is a reduced risk of adverse cardiovascular events and venous thromboembolism with FXI deficiency [ 13 , 16 , 24 – 26 ].…”