Purpose: To report the ocular findings in two siblings with IFAP and their mother and to review the natural course of the keratopathy of this disease.
Methods: Clinical ophthalmological examination of all patients and fundus photography of the carrier mother were performed.
Results: Both affected male children had severe photophobia, total superficial and deep corneal vascularization, and reduction of vision to counting fingers.The mother had tortuous retinal vessels.
Conclusions: Males with IFAP have an inexorable progression of corneal vascularization and loss of vision. Retinal vascular tortuosity may be another clinical sign of carrier status in females.