Bilateral persistent fetal vasculature: a study of 11 cases

To describe the clinical and radiologic features of patients with bilateral persistent fetal vasculature (PFV).

All patients diagnosed with symmetric, bilateral PFV over a 3.5-year period were included in this representative, observational case series. Only patients with more or less symmetrical disease in both eyes were included. Examination under anesthesia was performed in all but 1 case. B-scan ultrasonography of the eye was performed in all cases. A computed tomographic or magnetic resonance imaging scan was done if the ocular ultrasonography was inconclusive. A systemic workup was performed to rule out associated systemic anomalies.

The study group included 11 patients. Of the 22 eyes studied, 20 (91%) showed features of both the anterior and the posterior forms of PFV. Two children tested positive for IgM rubella antibodies. Ten eyes (45.5%) had a horizontal corneal diameter of less than 10 mm. Fifteen eyes (68.2%) had cataract at presentation. Intraocular pressure was >21 mm Hg in 17 eyes (77.3%).

Microcornea and cataract were common findings. Bilateral PFV cases were more commonly associated with combined anterior and posterior PFV. Bilateral PFV, although infrequent, should be considered in the differential diagnosis of bilateral leukocoria.