Related Experiment Video
Updated: Jun 14, 2025
Vessel-sparing Excision and Primary Anastomosis
Published on: January 7, 2019
Surgical Outcomes of Children with Unilateral Congenital Cataract and Persistent Fetal Vasculature
1Unit of Vitreoretinal Surgery, Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.
Insights
Surgical interventions for pediatric congenital cataract and persistent fetal vasculature (PFV) can achieve significant visual improvement. However, outcomes depend on the presence of anterior or posterior segment pathologies, highlighting the importance of careful case selection.
Area of Science:
- Ophthalmology
- Pediatric Surgery
- Genetics
Background:
- Congenital cataract and persistent fetal vasculature (PFV) are leading causes of pediatric visual impairment.
- Early diagnosis and surgical intervention are crucial for visual rehabilitation in affected children.
Purpose of the Study:
- To characterize pediatric patients with congenital cataract and PFV.
- To evaluate surgical interventions and visual outcomes in this cohort.
- To identify factors influencing visual prognosis.
Main Methods:
- Retrospective observational study of 82 children (birth to 15 years) undergoing surgery for congenital cataract and PFV (ICD-10 codes Q12.0 and Q14.0).
- Data collected included clinical history, ocular parameters (IOP, VA), surgical details, complications, and outcomes.
- Surgical procedures included lensectomy, vitrectomy, or combined approaches.
Main Results:
- Eleven children (6 months to 12 years) were analyzed for outcomes.
- 81.8% achieved at least light perception vision; 18.2% achieved hand motion vision.
- 55.6% achieved finger-counting vision or better; 18.2% had measurable Snellen acuity.
- One eye (9.1%) had complete vision loss; another developed sympathetic ophthalmia.
Conclusions:
- Absence of anterior segment pathology in congenital cataract with PFV correlates with better visual outcomes.
- Eyes with anterior or complex posterior segment pathology have a poorer visual prognosis.
- Careful patient selection is vital due to the risk of postoperative sympathetic ophthalmia; further research into PFV pathogenesis is needed.
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.
