VWD is a primary hemostasis deficiency presenting with mucosal bleeding, easy bruising, menorrhagia, and heavy bleeding during labor [8]. In the literature, several studies have reported about the safety and availability of conventional liver surgery in patients with ICD, including VWD [7, 9]. Furthermore, Denzer et al. reported that mini-laparoscopy-assisted liver biopsy was safe for patients with ICD [10]. None of the 61 patients had postintervention bleeding requiring reintervention [10]. Kobayashi et al. reported no significant difference in operative time, estimated blood loss, red blood cell transfusion rates, Clavien–Dindo classification 3–4 complications, or mortality rates between patients with and without ICD who underwent hepatectomy [7]. Yoshimoto-Haramura et al. reported that the postoperative complication rate did not increase in patients with ICD who underwent hepato-biliary-pancreatic surgery at qualified centers [9]. When limited to patients with VWD, several reports have described hepatectomies for patient safety [7, 12, 13]. Sato et al. reported a successful open hepatectomy in a patient with type 1 VWD patient [12]. However, their patient showed a preserved platelet count. Furthermore, type 2 VWD has been reported to cause more severe bleeding than type 1 [4]. Kobayashi et al. reported three successful cases of open hepatectomy for type 2 VWD; however, their cases had preserved platelet counts [7]. The clinical characteristics of the reported cases of VWD patients who underwent hepatectomy were presented in Table 2. In the present case, no preoperative findings suggested portal hypertension and thrombocytopenia was detected approximately 30 years ago. Therefore, the extremely low platelet count in the present case was considered to have been caused by type 2B VWD, which often coexists with thrombocytopenia [4]. Since the protocol of VWF/factor VIII administration in the present case was similar to that in previous reports [7, 12, 13], the extremely low preoperative platelet counts in the present patient might have caused the severe bleeding tendency.

In the current case, the most bleeding occurred at the retroperitoneum during mobilization of the right lobe due to inflammatory adhesions between the right liver and retroperitoneum, potentially caused by preoperative TACE. Careful consideration should be given to the indications for preoperative TACE of tumors located close to the surface of the liver. Although open conversion was considered intraoperatively, we considered that mobilization using a laparoscopic caudal view would have been more useful in the present case. Furthermore, open conversion may cause a greater amount of intraoperative bleeding from the abdominal wall and postoperative bleeding from the abdominal wall. Bleeding during parenchymal transection was not massive in the present case, which might be due to prior administration of frozen plasma during mobilization of the right liver. However, postoperative bleeding requiring intervention had occurred in the present case, and open conversion may have been a better approach.

The median intraoperative bleeding amount of LH (without other concomitant major operations) in our hospital from April 2016 to February 2024, when we standardized the LH procedure, was 100 mL (range, 0–2150 mL) in nonanatomical hepatectomies (n = 80) and left lateral sectionectomies (n = 9), and 200 mL (range, 0–1950 mL) in highly advanced anatomical hepatectomies (n = 23). The present case demonstrated the greatest intraoperative bleeding during LH at our hospital. Furthermore, we experienced no reoperation or interventional radiology due to postoperative bleeding in the LH, except in the present case. The above data are not an excuse for the clinical course of the present case; we concluded that a major part of the intra and postoperative bleeding in the present case might have been caused by the bleeding tendency of the patient. The patient in the present study also had refractory hematuria after transurethral biopsy for suspected prostate cancer 5 years before the hepatectomy, even with the appropriate administration of VWF/factor VIII. Furthermore, he experienced several episodes of gastrointestinal bleeding after polypectomy for colon polyps despite the appropriate administration of VWF/factor VIII.

In the present case, since omentum injury might have occurred outside the field of the laparoscopic view, more caution may be required for laparoscopic surgery. Notably, after abdominal exsufflation, untended bleeding can recur due to the discontinuation of pressure. Therefore, diligent hemostasis is a vital step in LH in patients with an ICD. Furthermore, the proper use of energy devices during surgery will prevent excessive bleeding and provide a better outcome. Nevertheless, postoperative bleeding may occur even with careful hemostasis in patients with ICD.

In conclusion, although we did not experience open conversion or reoperation, postoperative hemorrhage requiring percutaneous intervention occurred. Therefore, the indications for laparoscopic hepatectomy in patients with VWD should be carefully considered, and an open approach might still be standard for patients with VWD. Further studies are required to demonstrate the feasibility of LH in patients with VWD.