Lamellar hole-associated epiretinal membrane is a common feature of macular holes in retinitis pigmentosa

Abstract

Objective: To describe the features and surgical outcomes of macular holes (MHs) in patients with retinitis pigmentosa (RP).

Methods: A review of consecutive series of 110 patients (206 eyes) with RP who underwent comprehensive ophthalmic examinations was conducted. Eleven eyes of ten RP patients were identified with MHs (full thickness or lamellar). Atypical epiretinal membrane, which appeared on spectral-domain optical coherence tomography (SD-OCT) images as a thick homogenous layer of moderately reflective material that was present on the inner retinal layer, was considered to be lamellar hole-associated epiretinal proliferation (LHEP). Five eyes underwent modified vitreoretinal surgery, where hole margin LHEP tissue was retained.

Results: Nine eyes exhibited a lamellar macular hole (LMH), one exhibited a full-thickness macular hole (FTMH), and one exhibited both FTMH and LMH. LHEP was found in all eyes, identified intraoperatively as yellowish, sticky epiretinal membrane with internal limiting membrane beneath it. Two eyes experienced spontaneous closure of MHs without visual acuity (VA) improvement. Five eyes that underwent surgery achieved sealed MHs post-operatively and demonstrated improved, but limited, vision at their latest follow-up.

Conclusions: LHEP is common in MHs associated with RP. While some eyes could achieve spontaneous closure without any VA changes, a conservative vitreoretinal surgery approach, in which the hole margin LHEP tissue is spared, can effectively repair these MHs with limited VA improvement.

Fig. 1

Representative optical coherence tomography (OCT) images of lamellar hole-associated epiretinal membrane (LHEP) in macular holes in retinitis pigmentosa patients. a OCT scan shows a macular hole accompanied by macular detachment in the left eye of patient 1. LHEP appeared on OCT images as a thick, homogenous layer of moderately reflective material, present on the inner retinal layer (arrow). b OCT image of patient 6 shows a lamellar macular hole (LMH) with cystoid macular oedema (CMO) in the right eye; LHEP (arrows) was fused with condensed posterior cortical vitreous (arrowheads). Vitreoretinal traction is apparent. c OCT image of patient 5 demonstrates the LMH with LHEP (arrows); presence of a foveal bump (arrowhead) and wide intraretinal defects were noted

Fig. 2

a Ultra-wide scanning laser ophthalmoscopy (UWSLO) demonstrates peripheral salt–pepper like pigmentation in the left eye of patient 2. b, c Spectral-domain optical coherence tomography (SD-OCT) images show both full-thickness macular hole (MH) and lamellar MH in the left eye. Lamellar hole-associated epiretinal membrane (LHEP) (arrow) and foveal detachment were also detected. d Postoperative OCT image shows the MHs were closed and the fovea was attached in the left eye

Fig. 3

a, b Ultra-wide scanning laser ophthalmoscopy (UWSLO) demonstrates peripheral bone spicule pigmentation in both eyes of patient 3. c Spectral-domain optical coherence tomography (SD-OCT) images show lamellar macular hole (LMH) with wide intraretinal defects and foveal bump in the left eye. Lamellar hole-associated epiretinal membrane (LHEP) (arrows) was also detected. d Three years later, OCT image shows the LMH were closed in the left eye. LHEP remained (arrow)