Demir M Necati, Acar Mehmet Akif, Aral Yusuf Ziya, Unlü Nurten
Ankara Education and Research Hospital, Department of Ophthalmology, Ankara, Turkey.
Clin Ophthalmol. 2008 Jun;2(2):469-73.
To report a bilateral leukocoria case in a patient suffering from afibrinogenemia.
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.
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.
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.
报告一例患有无纤维蛋白原血症患者的双侧白瞳症病例。
介绍了一例先天性无纤维蛋白原血症患者出现双侧视网膜和玻璃体出血并接受玻璃体视网膜手术的观察病例。此外，还进行了全面的眼科和放射学检查以及玻璃体视网膜手术。
右眼完全恢复，而左眼出现严重的增殖性玻璃体视网膜病变且视网膜缩短。手术后三年的临床检查显示，右眼无晶状体，视网膜附着且眼内介质清晰，而左眼萎缩。
对于患有玻璃体或视网膜出血且无明显身体虐待迹象的婴儿，我们建议进行广泛的凝血检查。我们目前的病例进一步表明，无纤维蛋白原血症可能是双侧白瞳症发病机制的潜在原因，并且应提醒医生在几种出血易患因素中注意有无纤维蛋白原血症的存在。
