Proliferative Retinopathy in Full–Term Infants With Thromboembolic Risk Factors

Abstract

Abstract: : Purpose: To report the occurrence of proliferative retinopathy, mimicking severe retinopathy of prematurity, in full term babies with underlying thromboembolic risk factors. Methods:Retrospective review of case series managed at a pediatric retina referral center Results: We retrospectively reviewed the charts of two full term babies, who presented with severe proliferative retinopathy and vitreous hemorrhage. Both had immature retinal vascular development with peripheral retinal non–perfusion, mimicking a severe form of ROP. There was retinal vascular dragging and preripheral neovascularization. Unlike typical ROP, the neovascularization was not concentrated at a ridge and there was extensive hyperplasia of the retinal pigment epithelium. Both patients evolved to tractional retinal detachment in both eyes, and one developed angle closure and anterior neovascularization in both eyes. All four eyes underwent pars plana vitrectomy and repair of retinal detachments. Thromboembolic abnormalities included prothrombin 20210 mutation and factor V Leiden mutation in one patient, and perinatal ischemic stroke with low protein C and S and bilateral internal carotid and middle cerebral artery ischemia in the other patient. Conclusions: Severe proliferative retinopathy with abnormal vascular development can occur in the setting of perinatal thrombo–embolic events. We postulate that intrauterine ocular ischemia, or intrauterine metabolic acidosis in the setting of the various hematologic or vascular abnormalities adversely affects retinal vascular development in these patients. Ophthalmologic evaluation of patients with perinatal ischemic events should be performed as early as possible in order to allow peripheral ablation and prevent irreversible retinal ischemia and severe tractional retinal detachments.

Keywords: retinal neovascularization • ischemia • retinopathy of prematurity