Presentation
Bilateral leucocoria and visual impairment.
Patient Data
Patient aged 1 year. Indication: Bilateral microphthalmia and behavioral problems.
As clinically described, there is bilateral microphthalmia with increased density posteriorly in the right globe. On the left, there is the impression of a cord of increased density that traverses the posterior chamber.
MRI performed 1 year later. Indication: Viually handicapped with bilateral optic disc opacification.
As demonstrated at CT, there is bilateral microphthalmia. Cords can be seen between the posterior retina and the lens. In addition, there is high T1 and high T2 signal in the posterior portion of the right globe that does not suppress on fluid-suppression sequences. Features here are consistent with bilateral PHPV with associated retinal detachment on the right.
Case Discussion
There is bilateral PHPV with a cord extending from the posterior portion of the retina to the lens. On the right there are features of associated retinal detachment, which occurs in about 40% of cases.