Macular hole formation in patients with retinitis pigmentosa and prognosis of pars plana vitrectomy

Purpose: To investigate macular hole (MH) formation in patients with retinitis pigmentosa (RP) and to determine surgical prognosis for these patients. Methods: Data for four RP patients with MHs were retrospectively reviewed. Comprehensive ocular examinations, including electroretinography, Goldmann perimetry, color funduscopy, B-scan ultrasonic tomography, and optical coherence tomography (OCT), were performed. All patients underwent par plana vitrectomy followed by peeling of the posterior inner limiting membrane and gas tamponade. Preoperative best-corrected visual acuity ranged from 20/1000 to 20/100. Results: Typical full-thickness MHs were evident by OCT for three patients. Two of these patients had posterior vitreous detachment or vitreoretinal traction; one had cystoid macular edema in the fellow eye. A fourth patient had extensive retinal detachment; ophthalmoscopy revealed a hole in the macular region. After surgery, significant improvement of visual acuity was recorded for three patients; the patient with retinal detachment had no change in visual acuity in the affected eye. OCT showed adequate sealing of the MHs. Conclusions: We report results of a clinical investigation including four RP patients with concomitant MHs. Vitreoretinal surgery is necessary for effective management of MHs in RP.