Clinical history: Newborn with one eye relatively smaller than the other one. On examination the right eye was minimally smaller than left. B mode ultrasound and MRI images shown below.
There is a thin fibrous cord attached to the optic disc posteriorly; and posterior surface of the lens, anteriorly causing deformity of its shape. A relatively thick fibrous band is attached on its medial side just behind the deformed lens.Diagnosis: PHPV (persistent hyperplastic primary vitreous).Etiology: The exact cause is not known but patho-physiologically persistent hyperplastic primary vitreous is a rare congenital developmental anomaly of the eye that results following failure of the embryological, primary vitreous and hyaloid vasculature to regress. It can be present in three forms: purely anterior (persistent tunica vasculosa lentis and persistent posterior fetal fibrovascular sheath of the lens), purely posterior (falciform retinal septum and ablatio falcicormis congentia) and a combination of both.Management: if there is dens ambliopia on ophthalmological examination then conservative management is usually opted, but if not, then reconstructive surgery is an option. However the final decision depends on the expertise available and individual patient condition.Bakhtiar Ahmad, MD, FRCR, consultant radiologist, Medical Imaging Department, King Abdulaziz Medical City (National Guard Hospital), Saudi Arabia
