We report the case of a patient who was given a diagnosis of acquired factor XIII deficiency because of abnormal bleeding during bilateral sagittal split ramus osteotomy. He was referred to our hospital by a local dental clinic at the age of 16 years for surgical correction. Bleeding occurred during the extraction of a horizontally impacted third molar and bone drilling, but was stopped without difficulty. Bilateral sagittal split ramus osteotomy was planned 4 years after the first consultation. There was no history of bleeding in the patient or his family. A preoperative blood examination did not produce any abnormal findings. However, during the operation 1,220 cc of intraoperative blood loss was caused by difficulty in controlling bleeding. The bleeding was eventually controlled by applying pressure after the intravenous injection of tranexamic acid (1 g/10 mL). Five days after the operation, a close examination at our hospital’s Department of Hematology revealed a low factor XIII level (38%), which was indicative of factor XIII deficiency. Gradual osseous healing without wound healing failure or infection was observed. The subsequent measurement of the patient’s factor XIII level showed that it had slightly recovered, but was still low (56%). A diagnosis of congenital factor XIII deficiency was made, but the presence of inhibitory factors was excluded. When the bone-connecting material was removed with the patient under general anesthesia 1 year after the operation, continuous bleeding from the entire wound occurred, as was observed previously. The bleeding was difficult to control by applying pressure after the intravenous injection of tranexamic acid (1 g/10 mL), but was stopped by administering 480 IU of a freeze-dried human blood coagulation factor XIII concentrate (Fibrogammin® P).
