Pre- and postoperative OCT features and surgical outcomes of advanced retinitis pigmentosa with macular hole: case series and literature review

Abstract

Abstract Background Macular hole (MH) is a rare complication of retinitis pigmentosa (RP) and has an adverse impact on residual visual function. At present, the underlying mechanisms are not fully understood and surgical experience is limited. Methods We reviewed the medical records and optical coherence tomography (OCT) scans in a cohort of eight eyes of seven RP patients with MH in order to report their OCT features and vitreoretinal surgical prognosis. Results This study includes four lamellar macular holes (LMHs) and four full-thickness macular holes (FTMHs). Pre-operative OCT revealed other macular abnormalities in all eyes, such as epiretinal membrane (ERM), cystoid macular edema (CME), lamellar hole-associated epiretinal proliferation (LHEP) and vitreoretinal traction. MH progression and subjective vision worsening were noted in one LMH eye during a seven-month follow-up. All holes closed after vitrectomy with internal limiting membrane (ILM) peeling. At final follow-up, one eye had improved vision and seven eyes remained stable compared to baseline. Conclusions The occurrence of MH in RP is accompanied by various imaging characteristics, such as ERM, CME and LHEP, suggesting a multifactorial pathogenesis. Considering poor vision in most RP patients with potentially progressive MH, surgery appears to be effective in maintaining or improving the central vision in a period of time. Thus, vitrectomy should be performed as soon as possible and flap-assisted techniques or episcleral surgeries are needed for some special cases.

Keywords

• Macular hole
• Retinitis pigmentosa
• Optical coherence tomography
• Pars plana vitrectomy