Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
, 14, 112-116

Lamellar Hole-Associated Epiretinal Proliferation Presenting With Perifoveal Exudative Vascular Anomalous Complex

Case Reports

Lamellar Hole-Associated Epiretinal Proliferation Presenting With Perifoveal Exudative Vascular Anomalous Complex

Jakob Siedlecki et al. Am J Ophthalmol Case Rep.


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.


Fig. 1
Fig. 1
Multimodal imaging of perifoveal exudative anomalous vascular complex (PEVAC) and lamellar hole-associated epiretinal proliferation (LHEP). (A) Fundus photography, (B) near-infrared imaging, and (C) blue-autofluorescence revealed two small red (hyporeflective) dots in the temporal part of the fovea (arrows →). Blue-autofluorescence revealed alterations of the RPE (area surrounded by stars *). OCT (D–F) showed a lamellar macular hole (arrowhead ∇) with a hollow structure of medium reflectivity and a hyperreflective wall, highly suggestive of PEVAC; and intraretinal cavities (plus sign +) and LHEP (hash #) with intra-LHEP fluid (hollow circle ◦). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
Fig. 2
Fig. 2
Comparison of vessel imaging. By FAG (A–C), microvascular abnormalities with dilations and mild exudation can be detected on medium and late frames. One of the microvascular dilations also corresponds to hyperreflectivity on ICG (D–F). Microvascular abnormalities can be detected most precisely by OCT-A (G–I),. On the retina slab (G), the abnormal vessels (surrounded by a large circle) can easily be seen originating from the deep vascular plexus (I) and presenting with prominent microvascular dilations (arrows) located in the deep (I) and superficial vascular plexus (H). An area of capillary rarefication can be detected (dotted line) in the superficial capillary plexus slab (H). In the deep plexus (I), a microvascular loop can be seen temporal of the fovea (arrowhead).
Fig. 3
Fig. 3
Time course of retinal thickness on OCT. After three Ranibizumab injections, the thickness decreased from a maximum of 583 μm (A) to 472 μm (B), and the PEVAC lesion seemed partially to shrink. After a cessation of Ranibizumab treatment for three months, the thickness increased again to a maximum of 614 μm (not shown), driven by intra-LHEP cysts.

Similar articles

See all similar articles


    1. Witkin A.J., Ko T.H., Fujimoto J.G. Redefining lamellar holes and the vitreomacular interface: an ultrahigh-resolution optical coherence tomography study. Ophthalmology. 2006;113(3):388–397. - PMC - PubMed
    1. Parolini B., Schumann R.G., Cereda M.G., Haritoglou C., Pertile G. Lamellar macular hole: a clinicopathologic correlation of surgically excised epiretinal membranes. Investig Ophthalmol Vis Sci. 2011;52(12):9074–9083. - PubMed
    1. Pang C.E., Maberley D.A., Freund K.B. LAMELLAR HOLE-ASSOCIATED epiretinal proliferation: a clinicopathologic correlation. Retina. 2016;36(7):1408–1412. - PubMed
    1. Pang C.E., Spaide R.F., Freund K.B. Comparing functional and morphologic characteristics of lamellar macular holes with and without lamellar hole-associated epiretinal proliferation. Retina. 2015;35(4):720–726. - PubMed
    1. Pang C.E., Spaide R.F., Freund K.B. Epiretinal proliferation seen in association with lamellar macular holes: a distinct clinical entity. Retina. 2014;34(8):1513–1523. - PubMed

Publication types