Congenital Malformations of the Eye: A Pictorial Review and Clinico-Radiological Correlations

Figure 7

Persistent hyperplastic primary vitreous vs retinopathy of the prematurity. Persistent hyperplastic primary vitreous (a, b). The fundus examination of a one-year-old boy affected by Norrie’s disease shows persistent hyperplastic primary vitreous with the persistence of the hyaloid vessels and posterior membrane in the vitreous (a). A high-resolution T2WI sequence confirms the malformation and shows retinal detachment in the right eye (dotted white arrow in (b)) and bilateral presence of the so-called “martini glass sign” characterized by a triangular-shaped retrolental mass connected to the head of the optic nerve by a tubular image along the Cloquet canal (black arrows in (b)). In the left eye, there is a collapsed anterior chamber (white arrow in (b)) suggesting the coexistence of the anterior form of PHPV. Retinopathy of the prematurity in a newborn infant of 91 days, born at 27 weeks of gestational age and undergoing oxygen supplementation (c, d). Sagittal T2WI (c) and sagittal postcontrast T1WI (d) show retinal detachment appearing as folded membranes (white arrows in (c, d) with subretinal space fluid). A minimal hemorrhagic component is visible on the T1WI (black arrow in (c)).