抄録
Congenital factor VII deficiency is a rare hemorrhagic disorder with an autosomal recessive pattern of inheritance and incidence of approximately 1 in every 500000 live births. We report a case of sagittal splitting ramus osteotomy (SSRO) in congenital factor VII deficiency with mandibular prognathism.
The patient was a 20-year-old male. Congenital factor VII deficiency had been diagnosed during preoperative investigation for orthodontic surgery. The serum level of factor VII was 40%. We prepared fresh frozen plasma (FFP), recombinant factor VII, for abnormal bleeding during operation and prevention of delay in homeostasis while we were performing SSRO to set back the mandible. SSRO was done without recombinant factor VII and FFP, and hemostasis was achieved with the application of cold and continuous suction drainage. The amount of blood loss was 75ml. He was discharged on the 11th day after the surgery.
