This patient with past ocular history of leukocoria was referred forretinal evaluation. Ophthalmological evaluation under anesthesia was notable for a cataractous lens with spherical appearance,anterior segment dysplasia andpersistence of the iridohyaloid vessels and tunica vasculosa lentis. On further assessment with ultrasound the right eye was found to have a thin, vascularized membrane from the optic disc extending anteriorly. No retinal detachment, mass or calcificationwas appreciated. Anterior UBM confirmed the presence of an anteriorly rotated ciliary body with a thick retrolental membrane and a spherically shaped cataractous lens. The axial length was found to be 14.78 mm in the right eye and 17.6 mm on the left. Given the presence of advanced pathology, social factors and difference in size between the eyes it was determined thatsurgery was not indicated in this patient.Persistent Fetal Vasculature (PFV) previously known as PersistentHyperplastic Primary Vitreous is an heterogenous condition in which there is a failure of the fetal vasculature that supplies the lens in early stages to regress. The clinical manifestations seen in patients with PFV depends on the degree of non-regression and persistence of this abnormal vasculature. The evaluation of these patients requires a multimodal approach in order to be able to rule out threatening life conditions such as retinoblastoma, as well as to assess theseverity of the disease and visual prognosis. In general, surgery is not indicated for advanced pathology, such as severe optic nerve hypoplasia, severe retinal detachment, or microphthalmia, because postoperative vision is often poor in these cases. Additionally effective management of these patients requires motivated patents to ensure compliance with occlusive therapy and visual rehabilitation when indicated.
Presentation Date: 09/22/2022
Issue Date: 11/11/2022