Perioperative Management for a Patient with Severe Congenital Factor V Deficiency Undergoing Cochlear Implantation

Congenital factor V deficiency (CFVD) is a rare coagulation disorder, also known as para hemophilia, that was first described by Owren in 1947. Until now, no specific protocols for the management of these patients have been established, owing to the variable spectrum of bleeding manifestations. However, available research suggests that perioperative infusion of fresh frozen plasma (FFP) may aid in maintaining FV levels to prevent bleeding. We present the case of a 47-year-old woman with congenital FV deficiency who underwent cochlear implantation with no postoperative hemorrhagic complications.