Perioperative management of intrahepatic cholangiocarcinoma patients with hereditary coagulation factor V deficiency: a case report and literature review

BACKGROUND: Coagulation factor V (FV) is an essential factor to regulate and participate in the initial coagulation reaction, and it is the most important prothrombin activator in vivo. FV congenital deficiency is a rare hereditary disease, it exposes patients to hemorrhagic risk, with high morbi-mortality. Clinically, laparoscopic major hepatectomy has its own risk of intraoperative hemorrhage, and moreover, coupled with the lack of FV will increase the risk of bleeding even life-threatening. However, several studies have reported the cases of patients with FV deficiency undergoing surgery, there is no report of laparoscopic major hepatectomy in patients with FV deficiency so far. CASE DESCRIPTION: Here, we reported one case with no abdominal pain, nausea, vomiting, fever and other discomfort who was diagnosed with intrahepatic cholangiocarcinoma (IHC) complicated with hereditary FV deficiency and then received laparoscopic left hemihepatectomy after conversion therapy. After preoperative sufficient infusion of fresh frozen plasma (FFP) to improve coagulation function, careful intraoperative operation, and strict postoperative monitoring, no obvious complications occurred in the perioperative period. At present, the patient has an overall survival time of 18 months and is still followed up. CONCLUSIONS: It is suggested that laparoscopic major hepatectomy is safe and feasible for patients with hereditary FV deficiency under careful perioperative management.