“…5 Although the genes responsible for PFV are unknown, the lack of inheritance suggests that idiopathic, isolated, sporadic mutations may be responsible for the disease-a developmental defect caused by environmental factors during embryogenesis rather than genetic factors. 3 Ocular associations described for unilateral PFV include microphthalmia, megalocornea, Peters anomaly, cataract, colobomas, retinal detachment with retinal dysplasia, 6 intraocular cartilage, 7 and homonymous hemianopia. 8 Important systemic associations include hemiparesis, impaired coordination, ataxia, hypotonia, spastic quadriplegia, microcephaly and deafness, arachnoid cysts, 4 syndactyly, congenital heart anomalies, pulmonary atresia, asplenia, and Aicardi syndrome.…”