Surgical Treatment Of Challenging Cases With Persistent Fetal Vasculature

To present the surgical interventions and the visual rehabilitation of two cases of persistent fetal vasculature (PFV). The PFV (formerly persistent hyperplastic primary vitreous – PHPV) is usually a unilateral disease that can affect visual acuity differently: from a perfect visus to even light perception. If remains untreated, it can lead to severe amblyopia due to blocking the visual axis or to losing the eyeball due to recurrent intraocular hemorrhages, secondary glaucoma, and phthisis.

Two patients were operated on by the same surgeon at the Department of Ophthalmology, Albert Szent-Györgyi Health Centre, University of Szeged, Hungary

Case 1: A 3-year-old boy presented at our clinic with a fibrovascular stalk extending from the optic nerve to the anterior vitreous of the left eye which was diagnosed as posterior PFV, without affecting the lens. The visual acuity was 1,0 in his right eye, and 0,2 in his left eye. The fibrovascular stalk could be partially removed by pars plana vitrectomy having made the visual axis clean and relaxed the vitreoretinal traction. After intensive anti-amblyopic therapy, the visual acuity improved to 0,8. Due to the COVID-19 pandemic, we lost the patient for follow-up until 2022, when he presented with a cataract on the affected eye. He underwent a lens aspiration and in-the-bag IOL implantation, after that the VA improved to 0,7.

Case 2: in November 2022 a 3-year-old boy presented for further investigation and treatment with the diagnosis of total PFV. The fibrovascular tissue emerging from the optic disc on his left eye reached the posterior capsule of the lens. Due to obvious vitreoretinal traction, we decided to perform a combined cataract and pars plana vitrectomy surgery. His visual rehabilitation is still ongoing, but after 2 weeks when we started the anti-amblyopic treatment by patching the unaffected eye, he said that now he can see with his left eye, too.

The PFV involving the visual axis needs emergent surgical interventions. The goal of the surgical treatment is to clear the visual axis at the same time and to prevent the complications that probably occur during the natural course of the disease.