Bernard-Soulier症候群を有した下顎前突症患者の手術経験

概要

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Bernard-Soulier syndrome (BSS) is a rare hereditary autosomal disorder characterized by prolonged bleeding time, normal clot retraction and thrombocytopenia with large and abnormal platelets. A 20-year-old woman with mandibular prognathism, who had been treated orthodontically as a preoperative therapy for 16 months, was referred to us for sagittal splitting osteotomy of the mandibular rami. Since platelet count using automatic blood cell counter indicated severe thrombocytopenia (3, 000/mm3), the patient was examined in detail hematologically. The result disclosed BSS with normal platelet count (245, 000/mm3), normal clot retraction and large sized platelets which could not be measured by an automatic counter. For anesthesia, modified NLA was chosen and platelet retention rate was monitored during and after anesthesia because the alteration of the platelet function in BSS under anesthesia with vaporizing anesthetic agents, which can affect normal platelet function, is unknown. In addition, platelet-rich plasma was also prepared in advance. The surgery was carried out successfully and uneventfully.