AN EXPERIENCE OF PARTIAL NEPHRECTOMY IN A PATIENT WITH VON WILLEBRAND DISEASE

  • Ito Suguru
    The Department of Urology, Yamagata University Faculty of Medicine
  • Yamagishi Atsushi
    The Department of Urology, Yamagata University Faculty of Medicine
  • Kurokawa Masayuki
    The Department of Urology, Yamagata University Faculty of Medicine
  • Horie Shigemitsu
    The Department of Urology, Yamagata University Faculty of Medicine
  • Yagi Mayu
    The Department of Urology, Yamagata University Faculty of Medicine
  • Kurota Yuta
    The Department of Urology, Yamagata University Faculty of Medicine
  • Kanno Hidenori
    The Department of Urology, Yamagata University Faculty of Medicine
  • Sakurai Toshihiko
    The Department of Urology, Yamagata University Faculty of Medicine
  • Nishida Hayato
    The Department of Urology, Yamagata University Faculty of Medicine
  • Naito Sei
    The Department of Urology, Yamagata University Faculty of Medicine
  • Shibasaki Tomohiro
    The Department of Urology, Yamagata University Faculty of Medicine
  • Kawazoe Hisashi
    The Department of Urology, Yamagata University Faculty of Medicine
  • Ichiyanagi Osamu
    The Department of Urology, Yamagata University Faculty of Medicine
  • Kato Tomoyuki
    The Department of Urology, Yamagata University Faculty of Medicine
  • Nagaoka Akira
    The Department of Urology, Yamagata University Faculty of Medicine
  • Tsuchiya Norihiko
    The Department of Urology, Yamagata University Faculty of Medicine
  • Kabasawa Takanobu
    The Department of Pathology, Yamagata University Faculty of Medicine
  • Aizawa Keiko
    The Third Department of Medicine, Yamagata University Faculty of Medicine
  • Ishizawa Kenji
    The Third Department of Medicine, Yamagata University Faculty of Medicine

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Other Title
  • von Willebrand病患者に対する腎部分切除術の経験

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Abstract

<p>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.</p>

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