An Intrahepatic Cholangiocarcinoma Patient with von Willebrand Disease Successfully Treated with Robotic Hepatectomy under von Willebrand Factor Supplementation.

Introduction: Von Willebrand disease (VWD) is the second most common inherited coagulation disorder, and appropriate perioperative management is necessary when considering major surgery. There are few reports of patients with VWD who have undergone hepatectomy, especially minimally invasive hepatectomy. To our knowledge, this is the first reported case of a patient with VWD who successfully underwent robotic hepatectomy with von Willebrand factor (VWF) and factor VIII (FVIII) supplementation.

Case Presentation: A 75-year-old female was referred to our hospital because of a liver tumor that was diagnosed during follow-up after hepatitis C treatment. She had also been diagnosed with VWD in her 30s. CT and MRI showed a 24-mm mass in segment 8 of the liver, bordered by the middle hepatic vein (MHV). To ensure safe perioperative management, replacement therapy with a VWF- or FVIII-containing concentrate was administered from preoperative day 1 to POD 14. Robotic extended segmentectomy (segment 8) was performed, with resection of the MHV. Liver parenchyma was dissected using the crush and clamp technique under the Pringle maneuver. Estimated intraoperative blood loss was 160 mL, and total operative time was 601 min. The patient needed 2 units of fresh frozen plasma on POD 1; however, no other transfusions, including red blood cells, were required. Although the patient presented with postoperative ascites and was treated with diuretics, she was discharged on POD 20 without any bleeding event. The final pathological finding was intrahepatic cholangiocarcinoma.

Conclusions: We encountered a patient with intrahepatic cholangiocarcinoma and VWD who was successfully treated with anatomical hepatectomy by robotic-assisted laparoscopic surgery under perioperative replacement therapy with a VWF- or FVIII-containing concentrate. With appropriate perioperative management, major hepatectomy can be applied for VWD patients despite their high risk of postoperative hemorrhagic complications.