Case Report: Perifoveal Exudative Vascular Anomalous Complex in a Chinese Patient with Diabetes Mellitus

Perifoveal exudative vascular anomalous complex (PEVAC) is a recently described macular entity, which can be confused with other well-known vascular lesions such as retinal angiomatous proliferation. Perifoveal exudative vascular anomalous complex is more common than previously believed and is usually unresponsive to anti-vascular endothelial growth factor treatment. Clinicians should be aware of this disorder.

The purpose of this study was to report a case of PEVAC using multimodal imaging in a Chinese patient with diabetes mellitus but without diabetic retinopathy, followed by a brief review of the relevant literature.

A 53-year-old Chinese woman with a 7-year history of diabetes mellitus presented with complaints of a 1-month history of deterioration of visual acuity in her right eye. Complete ophthalmic examination, including fundus examination of the right eye, revealed an isolated lesion immediately temporal to the fovea, accompanied by small hemorrhages and small, hard intraretinal exudates. Fluorescein fundus angiography revealed a well-defined hyperfluorescent lesion in the early phase but with leakage in the late phase. Optical coherence tomography revealed an oval lesion with a hyperreflective wall and relatively dark lumen, intraretinal cystic spaces, and hard exudates. Two intravitreal injections of aflibercept resulted in reduced blood flow in the PEVAC lesion, but with more hemorrhaging and hard exudates and no improvement in visual acuity.

Perifoveal exudative vascular anomalous complex is an isolated, perifoveal, aneurysmal abnormality. It can occur in healthy patients, in addition to those with diabetes mellitus without retinopathy. In contrast to similar macular vascular anomalies, PEVAC does not typically respond to anti-vascular endothelial growth factor therapy.