Persistent Fetal Vasculature With Elongated Ciliary Processes in Children
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Published on: January 29, 2014
Nichelle Warren1, Rupal H Trivedi1, M Edward Wilson1
1Storm Eye Institute, Medical University of South Carolina, Charleston, South Carolina, USA.
Insights
Infants with unilateral persistent fetal vasculature (PFV) and stretched ciliary processes often require multiple surgeries and have poor visual outcomes. Visual axis opacification and glaucoma are common complications following initial surgery.
Area of Science:
- Ophthalmology
- Pediatric Ophthalmology
- Congenital Eye Abnormalities
Background:
- Unilateral persistent fetal vasculature (PFV) with elongated ciliary processes is a rare congenital eye condition.
- Outcomes and reoperation rates for this specific PFV subtype were previously understudied and excluded from major trials like the Infant Aphakia Treatment Study (IATS).
Purpose of the Study:
- To analyze preoperative measurements, reoperation rates, and postoperative complications in infants with unilateral anterior PFV and stretched ciliary processes.
- To provide data on surgical outcomes for this specific, less-studied group of PFV patients.
Main Methods:
- Retrospective case series design.
- Inclusion criteria: unilateral cataract surgery before 7 months, anterior PFV with elongated ciliary processes.
- Exclusion criteria: posterior retinal involvement, less than 6 months follow-up.
- Surgical procedures included lensectomy, posterior capsulectomy, and vitrectomy.
Main Results:
- Eleven eyes of 11 infants were analyzed, with a mean age at surgery of 2.4 months and average follow-up of 4.5 years.
- 91% were initially left aphakic; 27% later received secondary intraocular lenses (IOLs).
- Complications included visual axis opacification (60% of aphakic patients), glaucoma (18%), strabismus, and pupilloplasty. One eye was enucleated due to neovascular glaucoma and retinal detachment. Final visual acuity >20/200 in 18.2%.
Conclusions:
- Surgical outcomes for PFV with elongated ciliary processes are generally poor, with limited visual acuity potential.
- High rates of secondary surgeries are common, with visual axis opacification and glaucoma being significant postoperative concerns.
Purpose:
Outcomes and reoperation rates in infants with unilateral persistent fetal vasculature (PFV) with elongated or stretched ciliary processes have not been extensively studied and were excluded from the Infant Aphakia Treatment Study (IATS). The purpose of this study is to analyze the preoperative measurements, reoperation rates, and complications after surgery in unilateral anterior PFV with stretched processes.
Design:
Retrospective case series.
Methods:
Inclusion criteria consisted of unilateral cataract surgery prior to 7 months of age and anterior PFV with elongated ciliary processes. Eyes with posterior retinal involvement and less than 6 months of follow-up were excluded. All patients underwent lensectomy, posterior capsulectomy, and vitrectomy.
Results:
Eleven eyes of 11 patients were included. Patients had a mean age at surgery of 2.4 ± 1.4 months. Average follow-up was 4.5 ± 3.7years. Globe axial lengths were 18.6 ± 1.9 mm. Ten patients (91%) were initially left aphakic. Three patients (27%) later received a secondary intraocular lens (IOL), and 1 patient underwent an IOL exchange. Six out of 10 (60%) aphakic patients developed visual axis opacification. One aphakic patient required topical therapy for glaucoma. One additional patient developed neovascular glaucoma and retinal detachment. The eye was subsequently enucleated. Three patients underwent strabismus surgery. Two patients underwent pupilloplasty. Two patients (18.2%) had a final visual acuity better than 20/200.
Conclusion:
Eyes operated for PFV with elongated ciliary processes are unlikely to have a final visual acuity greater than 20/200 and many will need additional surgery. Postoperative visual axis opacification occurred in 60% and glaucoma developed in 18%.
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