Nasal hemophilic pseudotumor in a 2-year-old with recurrent epistaxis: a case report and review of the literature

Abstract

Abstract Hemophilic pseudotumor is a rare, yet dangerous complication of hemophilia. It has been reported previously at sites prone to recurrent trauma like long bones and pelvis. However, in the field of otorhinolaryngology, few cases are reported and therefore there is no established protocol for management. We hereby report a case of a 2-year-old boy, a known case of hemophilia A (factor VIII deficiency), who presented with recurrent epistaxis not responding to medical management. Imaging was done and revealed a heterogenous nasal mass compressing the left orbital wall, extending to the sphenoid sinus, and causing skull base erosion. The patient was successfully managed by evacuation and drainage of the pseudotumor via endoscopic endonasal approach and replacement of factor VII pre-and post-operatively. To our knowledge, this is the first case of nasal hemophilic pseudotumor managed by evacuation and drainage through an endoscopic endonasal approach, which was deemed successful.