Presentation
Bilateral leukocoria. History of prematurity and oxygen therapy.
Patient Data
Asymetrically small eye globes (microphthalmia) with loss of the normal high signal of the lens on T1 sequences. Bilateral intraocular retrolental soft tissue structure of intermediate signal intensity with subretinal hemorrhage of high signal intensity.
Cerebral volume loss with enlarged subarachnoud spaces and dilated ventricular system.
Case Discussion
The clinical presentation and MRI features are most consistent with retinopathy of prematurity with subretinal hemorrhage.
Retinopathy of prematurity (ROP), initially called retrolental fibroplasia is an ocular condition seen in the infant population. It occurs due to traction retinal detachment as a result of regression of the neovascularization secondary to the prolonged oxygen therapy. There is often a history of premature delivery, low birth weight, and/or prolonged ventilatory support.
The main differential diagnosis includes persistent hyperplastic primary vitreous which is characterized by a retrolental soft tissue of triangular shape appearing as low T2 signal extending from the head of the optic nerve to the posterior surface of the lens.