Bilateral simultaneous orbital hemorrhage and compartment syndrome as the presenting sign of acquired hemophilia A in an 11-year-old girl

A previously healthy 11-year-old girl presented with bilateral orbital compartment syndrome secondary to retrobulbar hemorrhages requiring emergency cantholysis. Four days earlier she had sustained head trauma without symptoms until her acute presentation. A basic hematologic profile was remarkable for a prolonged partial thromboplastin time, which did not completely correct with a 1:1 mixing study. This result raised suspicion for the most common acquired deficiency of clot stability, acquired factor VIII deficiency (acquired hemophilia A). Low factor VIII levels and the presence of autoantibodies against autologous factor VIII were confirmed. The child was treated daily with fresh frozen plasma and showed marked improvement over the ensuing days and weeks.