VPB1192 - A teenage boy with severe hemophilia A with inhibitors on Emicizumab prophylaxis presenting with hyphema

Background: Hyphema is an uncommon phenomenon in patients with hemophilia A which is associated with significant morbidity including blindness. Management of hyphema can be challenging due to a significant rebleeding rate even in individuals without bleeding disorders. To our knowledge, there is currently no data on the management of traumatic hyphema in severe hemophilia A with inhibitors on Emicizumab prophylaxis.

Aims: To describe a case of traumatic hyphema in a pediatric patient with severe hemophilia A with inhibitors on Emicizumab.

Methods: Case report

Results: 13-year-old male with severe hemophilia A and low titer inhibitor (peak 3.5 BU) on weekly Emicizumab prophylaxis (1.5 mg/kg) with good compliance presenting with left eye pain and vision loss 12 hours after left eye trauma from a Nerf gun bullet. Emicizumab prophylaxis was given six days prior. Patient was not infused with bypassing agent after the injury since he was asymptomatic. About 14 hours after trauma, patient became symptomatic and received rFVIIa (~100 mcg/kg) at home followed by another dose 4 hours later in the hospital. Ophthalmology evaluation revealed increased left eye intraocular pressure with grade IV hyphema requiring emergency anterior chamber washout and removal of anterior chamber fibrin clot via anterior vitrectomy (refer to Picture 1). The procedure was performed within 4 hours of hospital arrival and 20 hours after trauma. Aggressive management with rFVIIa was initiated resulting in excellent bleeding control and no rebleeding complications (refer to Table 1). Patient was discharged on daily rFVIIa on post-op day 4. Outpatient follow up 3 days after discharge showed normal intraocular pressure, no evidence of rebleeding and improving eye exam.

Conclusion(s): Timely management with surgery and aggressive use of bypassing therapy for trauma-related total unilateral hyphema successfully restored vision and prevented rebleeding in a pediatric patient with severe hemophilia A with inhibitors on weekly Emicizumab prophylaxis.