Presentation
Preterm birth, left leucocoria and microcornea.
Patient Data
Left orbit:
microphtalmos
a triangular-shaped retrolental soft tissue is seen extending from the posterior surface of the lens to the head of the optic nerve which appears as low T2 signal against the normal high T2 signal of the globe leading to the classic "Martini glass" appearance
a subretinal hemorrhage is noted of high signal on T1 and T2 that does not suppress on fluid-suppression sequences
Right orbit:
a linear sutructure is seen extending from the optic nerve head to the posterolateral surface of the lens wich is hypointense on T2 isointense on T1 with mild contrast enhancement representing persistent hyaloid artery in Cloquet's canal
The image illustrates the classic "Martini glass" appearance of PHPV
Case Discussion
MRI findings are reflective of persistent hyperplastic primary vitreous
complications of PHPV include retinal detachment, intraocular hemorrhage, rupture of lens capsule, cataract, secondary angle-closure glaucoma, traction retinal fold, and phthisis bulbi
differential diagnosis of PHPV includes retinoblastoma, retinopathy of prematurity, vitreoretinal dysplasia and ocular toxocariasis
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