Congenital fibrovascular pupillary membranes: clinical and histopathologic findings.
ABSTRACT To report the clinical and histopathologic findings associated with congenital fibrovascular pupillary membranes.
Seven infants were included, 6 with a unilateral congenital pupillary membrane and 1 with classic persistent fetal vasculature (PFV).
Patients underwent a membranectomy, pupilloplasty, or lensectomy. Histopathologic examination was performed on the excised membranes.
Visual acuity and pupil size.
Four of the 6 patients with a unilateral congenital pupillary membrane had 1 or more recurrences after a membranectomy and pupilloplasty. The most recent pupil size ranged from 2 to 5 mm in the affected eye. When last tested, the vision in the affected eye was excellent in 4 of the 6 patients. The 2 patients without recurrences of the pupillary membranes underwent multiple iris sphincterotomies at the time of the initial surgery. Histopathologic examination of 2 primary pupillary membranes showed fibrovascular tissue that did not stain for neuron-specific enolase. Smooth muscle actin was only present in vascular walls. In contrast, histopathology of a recurrent pupillary membrane revealed collagenized fibrovascular tissue that was immunoreactive for smooth muscle actin. Finally, histopathology of the retrolenticular membrane excised from an infant with classic PFV was similar to the latter aside from hypercellularity.
Congenital fibrovascular pupillary membranes in infants are likely a variant of PFV that may recur if incompletely excised. The risk of these membranes recurring may be reduced by excising as much as the membrane as possible and enlarging the pupil with iris sphincterotomies. A lensectomy should be avoided if possible.
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ABSTRACT: To report outcomes in cases of persistent hyperplastic primary vitreous (PHPV) and examine factors predictive of visual acuity in patients selected for surgery. Retrospective case series of 55 eyes in 50 patients presenting between 1990 and 2001 at the Children's Hospital, Westmead. 33 eyes underwent surgery aimed at visual rehabilitation. In surgical patients, median age at surgery was 77 days, and median age at final follow up was 28 months. Six (18%) achieved a visual acuity at final follow up of 6/60 or better and eight (24%) achieved acuity of less than 6/60 to counting finger vision inclusive. Those undergoing surgery before 77 days were approximately 13 times more likely to obtain a visual acuity of counting fingers or better than those operated later (p = 0.01). Neither posterior segment PHPV nor post-surgical glaucoma was a significant predictor of outcome in this selected group. Early surgery aimed at rehabilitation markedly improves the likelihood of useful visual outcome in selected eyes with PHPV.British Journal of Ophthalmology 08/2005; 89(7):859-63. · 2.90 Impact Factor
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ABSTRACT: A pupillary membrane in a case of congenital pupillary-iris-lens membrane with goniodysgenesis was surgically peeled from the lens without causing cataract formation. Histopathology revealed ectopic iris. The ectopic iris found in this condition differentiates congenital pupillary-iris-lens membrane with goniodysgenesis as an entity from persistent pupillary membrane, hereditary goniodysgenesis, and Rieger's anomaly. We suggest that congenital pupillary-iris-lens membrane with goniodysgenesis is a neurocristopathy. The finding of ectopic iris muscle is consistent with avian chimera experiments that have suggested that iris sphincter muscle is derived from the neural crest, not neural ectoderm. Membranes in this condition can be successfully removed when they cause vision loss and amblyopia.Ophthalmic surgery 25(9):580-3.
Article: Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV). LIV Edward Jackson Memorial Lecture.American Journal of Ophthalmology 12/1997; 124(5):587-626. · 4.22 Impact Factor