Surgical Repair of a Full-thickness Macular Hole in Retinitis Pigmentosa: a Case Report

Purpose: To report the long-term outcome after surgical repair of a full-thickness macular hole (FTMH) in a patient with retinitis pigmentosa (RP). Case summary: A 55-year-old male who had been diagnosed with retinitis pigmentosa in both eyes 5 years earlier presented with decreased visual acuity in his left eye over the last 6 months. On examination, his Snellen best-corrected visual acuity (BCVA) was 1.0 in the right eye and 0.3 in the left eye. Slit-lamp examination of the anterior segment was remarkable only for posterior chamber intraocular lenses in each eye. Fundus examination demonstrated extensive bony spicule-like pigmentation in the mid-peripheral region in both eyes and a FTMH with approximately one-third disc diameter in the left eye. The optical coherence tomography (OCT) findings confirmed a FTMH with a surrounding cuff of intraretinal fluid and vitreomacular traction in the left eye. The patient underwent 23-gauge pars plana vitrectomy (PPV) with indocyanine green-assisted internal limiting membrane peeling and gas tamponade. One week postoperatively, an anatomically well-sealed macular hole was confirmed by OCT. At the 3-month postoperative follow-up, the BCVA improved to 0.63 and the hole remained closed until his last follow-up (postoperative 6 years). Conclusions: Although macular hole is a rare occurrence in RP patients, it should be considered as a cause of significant visual loss in patients with this disorder. Our case suggested that over the long-term, PPV may be tolerable in the management for FTMH in RP.