Poster 2 Joaquin Castro Navarro

Advantages:

To report a case of unilateral recurrent macular hole in a patient affected by retinitis pigmentosa (RP).

Methods:

A 64-year-old woman, with the previous diagnosis of RP, was referred to our department with a complaint of central visual loss in her left eye (LE) for 12 months. A complete ocular exploration, including determination of Best Corrected Visual Acuity (BCVA) (decimal notation), fundoscopy and Optical Coherence Tomography (OCT) examination was performed.

Effectiveness / Safety:

At first examination, her BCVA was 0.1 in her LE, and 0.8 in her right eye (RE). An examination of the anterior segment was unremarkable. A fundoscopy examination of her LE revealed the presence of a stage-4 macular hole (MH) with a cuff of subretinal fluid surrounding it. A posterior vitreous detachment was observed. Narrowing of the retinal arterioles, waxy yellow appearance of the disk and hyperpigmentation in a bone-spicule configuration in the midperipheral retina were also observed in both eyes. However, the macular appearance of her RE was normal. An electroretinogram showed reduced scotopic and photopic responses in both eyes. Perimetry revealed the presence of an annular scotoma in both eyes, associated to central scotoma in her LE. OCT (Stratus OCT, Carl Zeiss) confirmed the presence of a full-thickness MH in her LE. Therefore, we decided to perform 20-gauge pars plana vitrectomy (PPV). Peeling of the internal limiting membrane (assisted by the dye Brilliant Blue G, Brilliant Peel ®) in the area around the macular hole and 25% sulphur hexafluoride intraocular gas fill were performed. Face down positioning for 5 days was also advised. At 2 months after the vitrectomy, her BCVA was 0.4 (decimal notation). Fundoscopy and OCT confirmed the hole closure, but severe macular atrophy was observed. After 6 months, her BCVA was 0.2. A biomicroscopy showed the presence of a posterior subcapsular cataract. Fundoscopy and OCT confirmed the closure of the MH. Therefore, crystalline extraction with phacoemulsification was performed, and no complications occurred. However, BCVA after cataract surgery did not improve. The patient came to our centre 2 years after PPV complaining of mild central visual acuity loss in her LE. Her BCVA at this time was 0.1 in the LE and 0.8 in the RE. A fundoscopy and OCT examination demonstrated the reopening of the previously closed full-thickness MH and the presence of a ring of subretinal fluid and intense retinal atrophy surrounding the hole. Because of the guarded prognosis, and the high likelihood that a reopening had occurred again due to the severe retinal atrophy around the hole, we decided not to perform a new surgery.

Conclusion:

The pathogenesis of macular hole formation in patients with retinitis pigmentosa is unclear. Surgical outcomes may not always be favourable, and the possibility of reopening must be taken into account, even after a long time.