Lamellar hole-associated epiretinal proliferation presenting with perifoveal exudative vascular anomalous complex

Spectral-domain optical coherence tomography (SD-OCT) commonly reveals lamellar-hole-associated epiretinal proliferation (LHEP) as an avascular homogenous layer of premacular material with medium reflectivity, as recently described in various traction maculopathies, mostly in lamellar macular holes (LMH). We have used multimodal imaging to examine a patient suffering from unilateral advanced atrophic LMH presenting LHEP with perifoveal exudative vascular anomalous complex (PEVAC) and intra-LHEP edema fluctuating under anti-vascular endothelial growth factor (anti-VEGF) therapy.

A 77-year-old male presented with decreased vision in the left eye attributable to longstanding LMH. He complained of worsening symptoms for six months. Whereas SD-OCT showed classic tractional epiretinal gliosis in the right eye, the left eye exhibited atrophic LMH and a significant amount of LHEP containing hyperreflective round lesions and hyporeflective cystoid spaces. Fluorescein/indocyanine green angiography demonstrated PEVAC with large anomalous vessels and exudation. OCT angiography revealed abnormal vessels originating from the deep retinal plexus. After anti-vascular endothelial growth factor (anti-VEGF) therapy, the intraretinal edema seemed to decrease.

Perifoveal exudative vascular anomalous complex can occur in eyes with advanced LMHs causing edema inside LHEP. Pathologic vessels appear to originate from the deep retinal plexus. Given that LHEP formation is proposed to be a glial-cell-driven process, Müller cells may play a decisive role in the pathogenesis of the presented vascular malformation. Because of spontaneous fluctuation of the associated edema, the role of anti-VEGF remains questionable, while a functional response to therapy might be limited according to the progressive atrophic lamellar defect with intraretinal tissue loss.