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Early vitrectomy effective for bilateral combined anterior and posterior persistent fetal vasculature syndrome
Mark K Walsh1, Kimberly A Drenser, Antonio Capone
1Associated Retinal Consultants PC, Royal Oak, Michigan 48073, USA. walshmk@gmail.com
Insights
Surgery for bilateral persistent fetal vasculature syndrome (PFVS) can preserve vision and prevent eye loss. Vitrectomy is beneficial for children with this rare condition, offering improved visual outcomes.
Area of Science:
- Ophthalmology
- Pediatric Retina
- Surgical Innovation
Background:
- Persistent fetal vasculature (PFVS) is a congenital eye anomaly.
- Bilateral cases with combined anterior and posterior involvement are rare and challenging.
- Limited data exists on surgical outcomes for this specific PFVS subtype.
Purpose of the Study:
- To review surgical outcomes in patients with bilateral combined anterior and posterior persistent fetal vasculature syndrome (PFVS).
- To evaluate the efficacy of vitrectomy in managing this rare condition.
Main Methods:
- Retrospective chart review of pediatric patients diagnosed with bilateral PFVS (1988-2008).
- Inclusion criteria: bilateral posterior PFVS, no family history or genetic testing for Norrie disease/FEVR.
- Surgical intervention involved vitrectomy with or without lensectomy.
Main Results:
- 22 patients with bilateral combined anterior and posterior PFVS were identified.
- Of 13 patients with visual acuity data, 69% maintained light perception vision.
- Of 28 operated eyes, only 11% became phthisical (lost).
Conclusions:
- Untreated bilateral PFVS with posterior involvement leads to poor visual prognosis.
- Vitrectomy is beneficial for bilateral combined anterior and posterior PFVS, preserving vision and preventing phthisis bulbi.
- This study highlights the positive impact of surgical intervention in a rare pediatric eye condition.
Purpose:
The purpose of this study was to review our surgical experience with patients with bilateral combined anterior and posterior persistent fetal vasculature syndrome (PFVS).
Methods:
We retrospectively reviewed the charts of all patients seen in our tertiary care pediatric retinal practice from 1988 to 2008 with a potential diagnosis of bilateral PFVS with posterior involvement. Clinical diagnosis required the presence of either bilateral persistent hyaloidal stalk tissue with retinal involvement or bilateral dense retrolental fibrovascular plaques (usually with no posterior view preoperatively) without a family history or genetic testing consistent with Norrie disease or familial exudative vitreoretinopathy.
Results:
Chart review showed 22 vitrectomized patients with clinical findings consistent with bilateral PFVS with posterior involvement who did not have a family history or genetic testing consistent with Norrie disease or familial exudative vitreoretinopathy. All 22 of these patients with posterior retinal involvement also had anterior findings and thus can be classified as combined anterior and posterior PFVS. Of the 13 patients with visual acuity follow-up data, 9 patients (69%) maintained at least light perception vision in at least 1 eye at last follow-up. Of the 28 operated eyes in 16 patients with follow-up data, 3 eyes (11%) were phthisical at last follow-up.
Conclusion:
Children with bilateral PFVS with posterior retinal involvement have a dismal visual prognosis if left unoperated. In this relatively large series of a rare condition, we find that vitrectomy with or without lensectomy is beneficial in bilateral combined anterior and posterior PFVS in two regards: maintenance or restoration of vision and avoidance of phthisis bulbi.
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