Hemorrhagic glaucoma in an infant with hemophilia, spontaneous hyphema, aniridia, and persistent iris vessels

In January 2000, a 2-month-old infant with known hemophilia A and aniridia presented with a new finding of spontaneous hyphema and severe intraocular pressure (IOP) elevation of the left eye. The IOP remained severely elevate d (44 mm Hg) after 48 hours of intensive medical management and factor Vm a dministration, An anterior chamber washout was performed followed by normal ization of IOP. During examination performed with the patient under sedatio n, an extensive capillary plexus was noted over the anterior lens of both e yes consistent with persistent iris structures associated with aniridia (Fi gure 1).(1,2) These vessels appeared to be the source of the spontaneous hy phema in the left eye (Figure 2).(3) To our knowledge, this is this first t ime that this association has been reported.