Bilateral leukocoria in infant with afibrinogenemia

Abstract

M Necati Demir1, Mehmet Akif Acar1, Yusuf Ziya Aral2, Nurten Ünlü11Ankara Education and Research Hospital, Department of Ophthalmology, Ankara, Turkey; 2Aydin Menderes University, Faculty of Medicine, Department of Pediatric Hematology, Aydin, TurkeyPurpose: To report a bilateral leukocoria case in a patient suffering from afibrinogenemia.Methods: An observational case where congenital afibrinogenemia was presented with bilateral retinal and vitreous hemorrhages that proceeded to vitreoretinal surgery was presented. In addition, complete ophthalmic and radiological examinations and vitreoretinal surgery were performed.Results: Right eye had a complete recovery while the left eye showed serious proliferative vitreoretinopathy and shortened retina. Three years after the surgery clinical examination showed that the right eye was aphacic with an attached retina and clear ocular media while the left eye was phtysic.Conclusion: We recommend broad clotting profile for infants suffering from vitreous or retinal hemorrhages with no obvious physical abuse. Our present case furthermore implies that afibrinogenemia can lie beneath the pathogenesis of bilateral leukocoria and should alert physician for the presence of an afibrinogenemia among several types of bleeding predispositions.Keywords: afibrinogenemia, retinal hemorrhage, vitreous hemorrhage, vitrectomy