Perioperative Management of Hemophilia A: An Uncommon Inherited Blood Disorder

Hemophilia A is a rare hematological disorder caused by an inherited X-linked factor VIII deficiency. It can be responsible for significant perioperative bleeding with fatal consequences if not diagnosed and managed correctly in the preoperative phase. We report the case of a 6-month-old infant scheduled for a cure for bilateral inguinal hernia. During the pre-anesthetic consultation, given that the child was uncircumcised and had not yet reached walking age, a blood workout was made, which revealed a prolonged aPTT with a patient/control aPTT ratio of 3.27, without hemorrhagic signs. An activity work-up of clotting factors showed less than 1% of normal levels of active clotting factor VIII. Thus, the diagnosis of a severe form of hemophilia A was made. Recombinant factor VIII was administrated in the preoperative period, with an activity factor VIII control over 60%. The surgical procedure was uneventful, with minimal intraoperative bleeding. Our case emphasizes the importance of prophylactic treatment and a proper pre-anesthetic assessment, which are essential to establish an appropriate management strategy and avoid complications, most of which are fatal.