Perioperative management of robotic nephrectomy in a child with severe hemophilia with high responding inhibitors: A case report

Hemophilia A is a congenital bleeding disorder. These patients often require factor VIII replacements predisposing them to the development of antibodies against factor VIII. Such patients require meticulous treatment strategies involving administration of Factor VII, FEIBA (Factor Eight Inhibitor Bypassing Activity) and factor VIII mimics like Emicizumab necessitating a multi-disciplinary team approach, careful titration and follow-up. These agents may also interfere with the coagulation assays rendering false normal values which can be detrimental to the perioperative management with increased risk of acute hemorrhagic episodes. There is scant literature on the management of perioperative bleeding in children with high titer antibodies on Emicizumab. We report the perioperative management of a 13 year old child with severe Hemophilia A with high responding inhibitors posted for robotic nephrectomy. This case highlights the need for a thorough preoperative workup, multi-disciplinary team approach, careful intraoperative titration and postoperative follow-up.