Early vitrectomy effective for bilateral combined anterior and posterior persistent fetal vasculature syndrome.
OBJECTIVE
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.


CONCLUSIONS
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.
Associated Retinal Consultants PC, Royal Oak, Michigan 48073, USA. walshmk@gmail.com
Mark K
MK
Walsh
Kimberly A
KA
Drenser
Antonio
A
Capone
Michael T
MT
Trese
D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D007839:Functional Laterality; D006801:Humans; D007223:Infant; D007908:Lens, Crystalline / Q000601:surgery*; D008297:Male; D054514:Persistent Hyperplastic Primary Vitreous / Q000503:physiopathology / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014792:Visual Acuity / Q000502:physiology; D014821:Vitrectomy; D014822:Vitreous Body / Q000601:surgery*