Purpose: To describe a new surgical technique and outcomes of lens-sparing vitrectomy and retrolental stalk dissection in posterior persistent fetal vasculature (PFV).

Design: Retrospective interventional case series.

Methods: RESULTS: Among the 21 included eyes, 8 (38%) had no macular involvement and 4 (19%) presented with microphthalmia. Median age at the first surgery was 8 months (range: 1-113 months). Surgical success was obtained in 71.4% of cases (15 of 21). In the remaining cases, the lens was removed because of capsular rupture in 2 cases (9.5%) and a large capsular opacity after stalk removal or an adherent stalk impossible to dissect in 4 cases (19.1%). In the capsular bag, IOL implantation was accomplished for all but 1 eye. None of the eyes developed retinal detachment or required glaucoma surgery. Endophthalmitis occurred in 1 eye. Secondary lens aspiration was needed in 3 eyes after a mean interval of 10.7 months following initial surgery. At last follow-up, half of the eyes remained phakic.

Conclusion: Lens-sparing vitrectomy is a useful approach to addressing the retrolental stalk in selected cases of persistent fetal vasculature syndrome. By delaying or avoiding lens extraction, this approach allows preservation of accommodation, reduction of the risk of aphakia, glaucoma, and development of secondary lens reproliferation.

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http://dx.doi.org/10.1016/j.ajo.2023.06.019DOI Listing

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Wagner Syndrome-like fundus presentation of atypical Persistent Fetal Vasculature.

Eur J Ophthalmol

January 2025

Department of Sciences, Ophthalmology Clinic, National Center of High Technology in Ophthalmology, Gabriele D'Annunzio University, Chieti, Italy.

Purpose: To report the case of a woman with atypical Persistent fetal vasculature (PFV) accompanied by more typical findings of Wagner Syndrome.

Results: A 58-year-old woman complained about persistent flashes and floaters for more than 6 months. Multimodal retinal imaging showed Bergmeister papilla and a fibrous gray strand floating behind the macula in the right eye.

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Article Synopsis
  • - Persistent fetal vasculature (PFV) is a congenital eye condition where the primary vitreous and hyaloid vessels fail to regress, causing abnormalities that can lead to cataracts and other complications.
  • - A novel surgical technique involving an intraocular endoscope was used safely in four cases of cataracts associated with PFV, allowing for better observation and treatment without significant complications.
  • - The use of the endoscope during surgery improved precision and effectiveness in managing the vitreous stalk and fibrovascular membranes, suggesting it as a valuable approach for PFV-related cataract treatment.
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Persistent fetal vasculature (PFV) is a rare ocular developmental disorder resulting from incomplete apoptosis of the embryonic hyaloid vasculature during the in-utero period. Variability in the development and regression of hyaloid vasculature is responsible for the wide range of clinical presentation of the disorder. PFV may manifest as anterior segment abnormalities (cataract, glaucoma, microphthalmia, elongated ciliary process with central traction, retrolental membrane, and shallow anterior chamber), posterior segment abnormalities (vitreous stalk, preretinal membranes, optic hypoplasia, and retinal folds), or with a combined anteroposterior disease.

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Purpose: To describe a new surgical technique and outcomes of lens-sparing vitrectomy and retrolental stalk dissection in posterior persistent fetal vasculature (PFV).

Design: Retrospective interventional case series.

Methods: RESULTS: Among the 21 included eyes, 8 (38%) had no macular involvement and 4 (19%) presented with microphthalmia.

View Article and Find Full Text PDF

Persistent fetal vasculature (PFV) syndrome is characterized by abnormal regression of the fetal hyaloid system and may occur in various forms. In this report, two atypical cases associated with posterior capsular defect and ectopic lens material located along Cloquet's canal are discussed. Ultrasonography of these patients presenting with bilateral total cataracts revealed a hyaloidal stalk extending from the optic nerve head to the retrolental area.

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