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Retinitis pigmentosa (RP) is a rare hereditary disease, yet it is the commonest cause of retinal dystrophy. Although lamellar hole-associated epiretinal membrane (LHEP) is commonly associated with macular holes, the development of macular holes in RP itself is rare. In this article, we report a rare case of bilateral LHEP in RP, and the surgical outcome of LHEP embedding and internal limiting membrane (ILM) flap in repairing a lamellar macular hole (LMH).
A 57-year-old woman who had RP with bilateral LHEP underwent a combination of cataract and vitrectomy surgery in her left eye. We preserved LHEP tissue and performed an ILM flap to avoid a iatrogenic full-thickness macular hole (FTMH) and facilitate LMH closure. Her right eye was monitored conservatively. At 2 weeks postoperative, the LMH in her left eye was anatomically repaired. There was limited improvement of visual acuity, which could be justified by disruption of the junction between the photoreceptors’ inner and outer segments (IS/OS junction) as evidenced by spectral-domain optical coherence tomography (SD-OCT).
The presence of LHEP in LMH is highly associated with disruption of the IS/OS junction and therefore patients should be counselled regarding guarded visual improvement post-vitrectomy. LHEP, which is derived from a Müller cell-driven process, is closely associated with LMH in RP, likely due to the progressive retinal tissue loss as a result from the disease nature of RP. Therefore, we suggest preserving LHEP tissue and performing an ILM flap as an improvised technique to avoid iatrogenic FTMH and facilitate LMH closure.