A 28-year-old male visited a nearby hospital with chief complaint of bilateral
back pain and
fever. He was diagnosed with a right complex renal
cyst (Bosniak classification, IIF) with a
kidney stone and was referred to our hospital. We first suspected an incarcerated
kidney stone and performed flexible transurethral lithotomy; however, his symptoms did not improve. Blood examination revealed prolonged APTT; subsequently, he was diagnosed with
von Willebrand disease (VWD). Because he experienced
pain due to the hemorrhagic renal
cyst, we performed partial
nephrectomy. Preoperatively, we supplemented the
von Willebrand factor (VWF) based on the VWF activity in the patient. Although intraoperative
bleeding was well controlled, he developed
bleeding from
pseudoaneurysms on the postoperative day (POD) 6. We immediately performed transarterial embolization along with VWF replenishment. VWF supplementation was discontinued on POD 14, and the patient was discharged on POD 23. Since then, he has not experienced a
bleeding recurrence or
pain. In patients with VWD, the perioperative administration of
desmopressin or VWF is recommended. Although several reports showed that surgeries involving these treatments are safe, only three cases with VWD, including the present case where the patient underwent partial
nephrectomy, have been reported. In the present case, postoperative
bleeding occurred despite exhibiting adequate perioperative VWF activity. Thus,
bleeding complications in patients with VWD undergoing partial
nephrectomy must be considered and should be carefully followed up.