Persistent Fetal Vasculature With Elongated Ciliary Processes in Children

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.