[Pigmented perivenous chorioretinal atrophy: Report of two cases]

Pigmented perivenous chorioretinal atrophy is a rare condition, the etiology of which is still unknown, with approximately one hundred cases described in the literature. The lesion is characterized by chorioretinal atrophy with retinal pigment in a bony spicule distribution along the retinal veins. These pigmentations are small round lesions, which are in a pattern similar to the appearance of osteoblastic cells. This condition is often bilateral and asymmetric. It appears to be sporadic most of the time. The atrophic areas do not affect the macula; therefore visual acuity is not disturbed. Optical coherence tomography shows an alteration of the external layers of the perivenous retina. Fluorescein angiography reveals a hyperfluorescence of the perivenous retina. Visual field measurement reveals a paracentral scotoma. This condition appears to be localized and self-limited, yet our knowledge is still limited because of the small number of cases reported. The present work reports two cases of pigmented perivenous chorioretinal atrophy in children. Our descriptions correspond to the data reported in the literature.