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Congenital fibrovascular pupillary membranes: clinical and histopathologic findings
Scott R Lambert1, Edward G Buckley, Phoebe D Lenhart
1Department of Ophthalmology, Emory University, Atlanta, Georgia 30322, USA. slamber@emory.edu
Insights
Congenital fibrovascular pupillary membranes may recur after surgery. Complete excision and iris sphincterotomies can reduce recurrence risk in infants, while lensectomy should be avoided.
Area of Science:
- Ophthalmology
- Pediatric Ophthalmology
- Histopathology
Background:
- Congenital pupillary membranes can impair vision in infants.
- Persistent fetal vasculature (PFV) is a known cause of congenital eye abnormalities.
Observation:
- Seven infants with congenital fibrovascular pupillary membranes were studied.
- Recurrence was observed in 4 of 6 infants after initial surgery.
- Histopathology revealed fibrovascular tissue, with recurrent membranes showing collagenization and smooth muscle actin.
Findings:
- Congenital fibrovascular pupillary membranes may be a variant of PFV.
- Incomplete excision is linked to membrane recurrence.
- Iris sphincterotomies and complete membrane removal appear to reduce recurrence.
Implications:
- Surgical strategies should focus on complete membrane excision and pupil enlargement.
- Avoid lensectomy when managing these membranes.
- Understanding the histopathology aids in predicting and managing recurrence.
Purpose:
To report the clinical and histopathologic findings associated with congenital fibrovascular pupillary membranes.
Design:
Case series.
Participants:
Seven infants were included, 6 with a unilateral congenital pupillary membrane and 1 with classic persistent fetal vasculature (PFV).
Methods:
Patients underwent a membranectomy, pupilloplasty, or lensectomy. Histopathologic examination was performed on the excised membranes.
Main Outcome Measures:
Visual acuity and pupil size.
Results:
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.
Conclusions:
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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