Congenital cataract with persistent fetal vasculature without anterior segment pathology associated with best postoperative visual acuity: Study
Congenital cataract with persistent fetal vasculature (PFV) represents a complex and challenging condition in paediatric ophthalmology. PFV, formerly known as persistent hyperplastic primary vitreous, is a rare developmental anomaly characterized by incomplete regression of the primary vitreous and embryonal hyaloid vasculature, leading to a spectrum of ocular abnormalities, including cataract and retinal detachment. In the worst case, the persistent hyaloidal stalk is integrated anteriorly into the posterior lens capsule and posteriorly to the vascular bundle of the optic nerve head (ONH) and exerts tractional forces radially in all directions. Ultimately, tractional retinal detachment (TRD) may occur due to retrolental fibrovascular tissue proliferation and contraction. In PFV eyes, concomitant retrolental stalk, avascular peripheral retina, and regional capillary dropout have been observed with fluorescein angiography examination.
The management of unilateral congenital cataract with PFV poses unique therapeutic challenges. Surgical intervention is often required to address the congenital cataract and associated PFV-related anterior and/or posterior segment complications, with techniques such as lensectomy, vitrectomy, membranectomy, and retinal detachment repair. And in many cases multiple surgical interventions are needed. However, in cases with advanced pathology, including ONH hypoplasia, severe tractional retinal detachment, or microphthalmia, surgery is generally not a preferred choice since post-operative vision is often modest. Postoperatively, achieving optimal visual outcomes in the PFV cases can be hindered by factors including amblyopia, nystagmus, glaucoma, proliferative vitreoretinopathy (PVR), and refractive errors. Case selection for surgical treatment is of great importance, since long-standing complications such as sympathetic ophthalmia, ie, bilateral granulomatous panuveitis, may occur postoperatively and need to be considered.
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