Epiretinal Proliferation Associated with Macular Hole and Intraoperative Perifoveal Crown Phenomenon

Abstract

PurposeTo discuss the unique morphology and origin of epiretinal proliferation associated with macular hole (EPMH) occasionally observed in full-thickness macular hole (FT-MH) or lamellar hole (LH) and to introduce the perifoveal crown phenomenon encountered when removing this unusual proliferative tissue.MethodsSixteen patients showing EPMH in spectral domain-optical coherence tomography were selected from 212 patients diagnosed with MH, LH, FT-MH, impending MH, macular pseudohole, or epiretinal membrane between January 2013 and December 2014. Of the 212 patients included for clinical analysis, 33, 23, 11, 7, and 190 exhibited LH, FT-MH, impending MH, macular pseudohole, and epiretinal membrane, respectively. We reviewed visual acuity, macular morphology, and clinical course. Surgical specimens were analyzed histologically.ResultsEPMH presented as an amorphous proliferation starting from the defective inner/outer segment (IS/OS) junction covering the inner macula surface. Among the 16 patients with EPMH, 11 underwent vitrectomy, and all exhibited the intraoperative perifoveal crown phenomenon. EPMH tissue was sampled in three patients, one of whom had more tissue removed than intended and showed delayed recovery in visual acuity. Despite hole closure, IS/OS junction integrity was not successfully restored in four of 11 patients. Five patients were followed-up without surgical intervention. Visual acuity slightly decreased in three patients and did not change in one patient, while the remaining patient was lost during follow-up. Among the three perifoveal crown tissues obtained, two were successfully analyzed histologically. Neither tissue showed positivity to synaptophysin or S-100 protein, but one showed positivity to cytokeratin protein immunohistochemical staining.ConclusionsEPMH exhibited a distinct but common configuration in spectral domain-optical coherence tomography. An epithelial proliferation origin is plausible based on its configuration and histological analysis. Perifoveal crown phenomenon was observed when removing EPMH during vitrectomy.

Epiretinal Proliferations Associated with Lamellar Macular Holes: Clinical and Surgical Implications

“…Moreover, dell’Omo et al [17] did not find baseline BCVA to be a predictor of final visual outcome. Another study identified pan-keratin, a component of RPE cells, as a component of LHEP [15]. This study hypothesized ellipsoid disruption to be a bridge for RPE cells to migrate and form LHEP.…”

“…In our LMH population, LHEP was found on the SD-OCT of 53.2% of the evaluated eyes, the highest prevalence reported so far. In previous studies, LHEP prevalence ranged between 20 and 44% [4, 11-13, 15]. …”

“…However, epiretinal proliferations (LHEP), a newly identified structure in LMH previously known as “dense ERM,” have been drawing attentions since they were first described [2, 5, 11]. The natural evolution of retinal imaging, namely spectral-domain (SD) OCT systems, allowed for a detailed evaluation of this clinical entity, reportedly present in 20–44% of all eyes with LMH [4, 11-15]. LHEP presents on SD-OCT as an intermediate reflective material on the edge of a ruptured fovea, mainly in LMH but also in full-thickness macular holes (FTMH) [11].…”

“…LHEP presents on SD-OCT as an intermediate reflective material on the edge of a ruptured fovea, mainly in LMH but also in full-thickness macular holes (FTMH) [11]. During surgery, it may be noticed as a yellow elastic jelly that may toughen ERM and internal limiting membrane (ILM) peeling, an effect recently named “perifoveal crown phenomenon” [15]. Its yellowish color has a likely connection with a xanthophyll pigment identified in histologic analysis [5].…”

“…In spite of the absence of a clear etiology for LHEP origin, the most likely theory is that it results from the migration of retinal Muller glial cells [11], to hypothetically allow for a “physiologic” closure of the LMH. Another study hypothesized its origin in the retinal pigment epithelium (RPE), after it stained positive to pan-keratin, a marker of these cells [15]. …”