Surgical Outcomes of Children with Unilateral Congenital Cataract and Persistent Fetal Vasculature

Purpose: To delineate the characteristics, surgical interventions, and visual outcomes among children diagnosed with congenital cataract and persistent fetal vasculature (PFV). Patients and Methods: Retrospective observational single-center study was conducted between January 1, 2009, and December 31, 2019, at Helsinki University Hospital. The national cohort encompassed 82 children aged from birth to 15 years who underwent lensectomy, 3-port vitrectomy, or a combined procedure, with the objective of achieving visual rehabilitation. Among the surgical cohort, paediatric cases with International Classification of Disease (ICD-10) codes Q14.0 for PFV and Q12.0 for congenital cataract were identified and analyzed. Data were collected through a comprehensive review of medical records, encompassing clinical history (birth weight), gender distribution, ocular parameters (laterality, intraocular pressure [IOP], visual acuity [VA]), details of cataract and vitreoretinal surgical interventions, indications for surgery, postoperative ophthalmic complications, as well as evaluations of functional and anatomical outcomes. Results: The cohort consisted of 11 children, ranging in age from 6 months to 12 years. Surgical intervention resulted in the attainment of at least light perception vision in nine of the operated eyes, representing 81.8% of cases. Among these, two eyes (18.2%) achieved hand motion vision, while 5 eyes (55.6%) achieved vision of finger counting or better. Additionally, two eyes (18.2%) achieved visual acuity measurable on the Snellen chart. However, one eye (9.1%) experienced complete vision loss, while the contralateral eye developed sympathetic ophthalmia. Conclusions: Congenital cataract with PHV without anterior segment pathology associated with best postoperative VA, while eyes with anterior segment pathology or a complex posterior segment pathology had poor visual prognosis. Case selection for surgery is considered of great importance, since sympathetic ophthalmia may occur postoperatively. Understanding more deeply pathogenesis of PFV is warranted.