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Jackson F. Lever, Caesar Luo, Annahita Amireskandari, Kimberly Drenser; Persistent Fetal Vasculature Syndrome: Unilateral or Bilateral?. Invest. Ophthalmol. Vis. Sci. 2012;53(14):941.
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Describe the contralateral findings in presumed unilateral persistent fetal vasculature syndrome (PFVS) through clinical examination and fluorescein angiography in one institution.
Retrospective case series of patients presenting to a single institution between 2007-2010 with PFVS as final diagnosis. Outcomes included patient demographics, clinical findings in both eyes, and fluorescein angiographic interpretations in both eyes.
All patients referred to Associated Retinal Consultants with the diagnosis of PFVS were analyzed. 41 patients were identified. 25 (61%) were male and 16 (39%) were female. The average age was 11.6 months, with a range from 3 days to 8 years. 12 patients (29.3%) demonstrated bilateral PFVS and were excluded. Of the remaining 29 patients with unilateral PFVS, 11 patients underwent bilateral fluorescein angiography. Of these 11 patients, all uninvolved eyes were reported by clinical exam to be normal. 6 of these patients (54.5%) showed abnormal fluorescein angiographic findings, including abnormal foveal avascular zone with blunted capillary buds (n=4), incomplete peripheral vascularization or capillary dropout (n=4), and peripheral arteriovenous shunting (n=1).
PFVS has been regarded as primarily unilateral in approximately 90% of patients. Our series demonstrates that bilateral angiographically evident abnormalities may be higher than previously reported. Long term clinical relevance of these findings remains to be seen. This series may also serve to further enhance the normative database of fluorescein angiography and elucidate pathologic vs. normal developmental angiographic changes.
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