J Dent Anesth Pain Med.  2015 Mar;15(1):25-29.

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

• Lee SE
• Choi YJ
• Chi SI
• Kim HJ
• Seo KS

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 (ROTEMâ„¢) and noninvasive measurements of total hemoglobin (SpHb) using Masimo Radical 7 (Masimo Co. CA, USA).

Keyword

Continuous noninvasive hemoglobin monitoring; Factor XI deficiency; Orthognathic surgery; Rotation thromboelastometry

MeSH Terms

Anesthesia*
Factor XI Deficiency*
Factor XI*
Hemorrhage
Humans
Orthognathic Surgery*
Osteotomy
Thrombelastography
Factor XI

Figure

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  Fig. 1 Perioperative anesthetic monitoring of vital signs. ETCO2: the level of carbon dioxide released at the end of expiration, SpO2: pulse oximetry oxygen saturation, systolic: systolic blood pressure, Mean: mean blood pressure, diastolic: diastolic blood pressure.

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  Fig. 2 Perioperative hemoglobin change. Total hemoglobin was continuously measured by the Masimo Radical-7. FFP: Fresh Frozen Plasma, Auto: Autotransfusion, ROTEM: Rotation Thromboelastometry.

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  Fig. 3 Thromboelastograph curve. The curve demonstrates clot initiation, propagation, stabilization, and lysis. CT: Clotting Time; CFT: Clot formation time; alpha: Alpha-angle; A10: Amplitude 10 min after CT; MCF: Maximum clot firmness; LI30; Lysis index 30 min after CT, ML; Maximum lysis.

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