A case report on a rare posterior type of persistent fetal vasculature

Abstract

Persistent fetal vasculature (PFV) is a rare congenital developmental malformation of the eye. It is caused by the incomplete regression of the fetal hyaloid vasculature. It is a rare and sporadic disease with an overall prevalence of 0.064%. Persistent fetal vasculature, previously referred to as persistent hyperplastic primary vitreous (PHPV), is usually characterized by microphthalmia, a shallow anterior chamber, elongated ciliary processes, a posterior subcapsular cataract and a fibrovascular stalk that extends from the optic disc to the lens.This case presented with a history of poor vision on the right eye. On ophthalmologic examination, no light perception was noted in the affected eye. Slit-lamp findings for both eyes were unremarkable. There was no note of microphthalmia, shallow anterior chamber nor was there a subcapsular cataract. On dilated funduscopy of the affected eye, a fibrovascular stalk was seen emanating from the optic nerve. This was further documented with ocular ultrasound. Based on the clinical findings, a diagnosis of persistent fetal vasculature, posterior type, was made. Since the affected eye had no visual potential, a conservative mode of management was instituted. Persistent fetal vasculature is a rare and sporadic disease, which usually presents with microphthalmos, microcornea and leukocoria. Isolated posterior forms of PFV only accounts for 11% of persistent fetal vasculature cases in the literature.This documented case of a solely posterior type of persistent fetal vasculature, could somehow contribute to the small amount of information on this diseases entity. This, in turn, would eventually enable us to identify and formulate a more successful management strategy