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

Jakob Siedlecki; Efstathios Vounotrypidis; Denise Vogt; Armin Wolf; Siegfried G Priglinger; Ricarda G Schumann

doi:10.1016/j.ajoc.2019.03.008

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

Am J Ophthalmol Case Rep. 2019 Apr 4:14:112-116. doi: 10.1016/j.ajoc.2019.03.008. eCollection 2019 Jun.

Authors

Jakob Siedlecki¹, Efstathios Vounotrypidis¹, Denise Vogt¹, Armin Wolf¹, Siegfried G Priglinger¹, Ricarda G Schumann¹

Affiliation

¹ Department of Ophthalmology, Ludwig-Maximilians-University Munich, Mathildenstr. 8, 80799, Munich, Germany.

Abstract

Purpose: 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.

Observation: 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.

Conclusions and importance: 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.

Keywords: Lamellar macular hole; Lamellar-hole-associated epiretinal proliferation; Perifoveal exudative vascular abnormality; Premacular proliferation.

Publication types

Case Reports