Orthognathic Surgery Using 1-Desamino-8-D-Arginine Vasopressin (DDAVP) in a Patient With Von Willebrand Disease: A Case Report

Von Willebrand disease (vWD) is a bleeding disorder with autosomal-dominant inheritance caused by quantitative and qualitative abnormalities in von Willebrand factor (vWF), which plays a role in primary hemostasis. In such cases, it is necessary to evaluate hemostatic function prior to surgery and take appropriate measures to manage bleeding. There are few reports on orthognathic surgery, which is a highly invasive procedure in the oral and maxillofacial regions, and all of them use blood products. We report a case of orthognathic surgery using 1-desamino-8-D-arginine vasopressin (DDAVP), a synthetic antidiuretic hormone, in a patient with vWD for jaw deformity. We report a case of orthognathic surgery using DDAVP, a synthetic antidiuretic hormone, in a patient with vWD for jaw deformity. A 26-year-old Japanese female visited another clinic with a chief complaint of mandibular prognathism and started preoperative orthodontic treatment for jaw deformity. Preoperative screening and examinations revealed prolonged APTT and decreased coagulation factor activity. The patient was referred to the hematology department at our hospital and diagnosed with type Ⅰ vWD (quantitative reduction in vWF). The patient was referred to our department for orthognathic surgery, and LeFort I osteotomy and bilateral sagittal split ramus osteotomy were planned to improve occlusion. The patient underwent a challenge test using intravenous injection of DDAVP in the hematology department to establish a response to DDAVP prior to surgery. As a result, an increase in vWD antigen levels and vWF activity was confirmed, and surgery was performed using DDAVP. There were no perioperative complications, and the patient’s perioperative course was uneventful.