Korean J Ophthalmol.  2016 Dec;30(6):399-409. 10.3341/kjo.2016.30.6.399.

Epiretinal Proliferation Associated with Macular Hole and Intraoperative Perifoveal Crown Phenomenon

• Son G
• Lee JS
• Lee S
• Sohn J

• KMID: 2373997
• DOI: http://doi.org/10.3341/kjo.2016.30.6.399

Abstract

PURPOSE
To 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.
METHODS
Sixteen 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.
RESULTS
EPMH 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.
CONCLUSIONS
EPMH 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.

Keyword

Epiretinal proliferation; Lamellar hole; Macular hole; Perifoveal crown phenomenon; Retinal pigment epithelium

MeSH Terms

Aged
Epiretinal Membrane/*diagnosis/etiology/surgery
Female
Follow-Up Studies
Fovea Centralis/*diagnostic imaging
Humans
Intraoperative Period
Male
Middle Aged
Retinal Perforations/complications/*diagnosis/surgery
Retrospective Studies
Tomography, Optical Coherence
Visual Acuity
*Vitrectomy

Figure

• 

  Fig. 1 Preoperative spectral domain-optical coherence tomography images of full-thickness macular hole (A,B), impending macular hole (C,D), and lamellar macular hole (E,F); (A) patient no. 10, (B) patient no. 7, (C) patient no. 8, (D) patient no. 6, (E) patient no. 3, and (F) patient no. 5. Note the thick proliferative tissue mounted at the edge of the hole (bold arrows). Eyes with full-thickness macular hole or impending macular hole were regarded to have inherent disruption of the inner/outer segment junction of the photoreceptor layer. Eyes with lamellar macular hole (E,F) are considered to have defective inner/outer segment junction when an ellipsoidal defect or disconnection is observable (bold triangle).

• 

  Fig. 2 Perifoveal crown phenomenon observed intraoperatively (patient no. 5). The internal limiting membrane is about to be peeled off with a retinal forceps. The perifoveal crown tissue is then preserved.

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  Fig. 3 Consecutive sections of preoperative macular configuration at a single instance in two patients; patient no. 5 (A,B,C,D) and patient no. 12 (E,F,G,H). Successive sections enable us to identify anatomical continuity of epiretinal proliferation associated with macular hole to the defective base of the lamellar macular hole (outlined manually by the translucent white line). Note that epiretinal proliferation associated with macular hole tissue appears to creep out of a defective outer retina and proliferate beyond the edge of the hole.

• 

  Fig. 4 Histological analysis of perifoveal crown tissue acquired from patient no. 1 (A) and patient no. 3 (B-D). (A) Hematoxylin and eosin stain staining of paraffin-embedded perifoveal crown tissue from patient no. 1. Aggregation of lymphocytes and cells with medium-density nucleus are scattered. (B) Hematoxylin and eosin staining of paraffin-embedded perifoveal crown tissue from patient no. 3. (C) Synaptophysin staining of perifoveal crown tissue from patient no. 3. No specific staining is observed. (D) Pan-keratin staining of perifoveal crown tissue from patient no. 3. Note cells with brownish-stained cytoplasm scattered throughout the specimen.

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