Two years outcomes of treating full-thickness macula hole associated with idiopathic macular telangiectasia type 2 by internal limiting membrane inverted flap technique

Abstract

Rational: Macular telangiectasia (MacTel) is an uncommon ocular disorder that can lead to legal blindness. MacTel type 2 is characterized by a bilateral loss of macular transparency, the presence of white crystals on the retina, aberrant blood vessel growth, and neurodegeneration of the macula. Full-thickness macular holes (FTMHs) are a prominent cause of vision reduction in MacTel type 2, and the standard care for an FTMH is pars plana vitrectomy (PPV) to restore the FTMH and best-corrected visual acuity (BCVA). However, surgical outcomes in previous reports were not good, with a lack of closure or a reopening of the FTMH, compared with those with an idiopathic FTMH. Thus, this study aimed to determine the surgical outcomes of PPV with the inverted ILM flap technique for the treatment of FTMHs with a 2-year postoperative follow-up in three patients with MacTel type 2. Patient concerns: This study involved 3 patients who had been diagnosed with MacTel type 2 at a local eye clinic and who was subsequently referred to our department for a more detailed examination. Diagnoses: Three patients were diagnosed with MacTel type 2 using dilated ophthalmoscopy, fluorescein angiography, and optical coherence tomography (OCT) in both eyes. A FTMH was developed and visual acuity decreased during follow-up period in all of the patients. Interventions: Each patient underwent PPV in 1 eye using the inverted ILM-flap technique, gas tamponade, and prone positioning. Outcomes: The FTMH was successfully closed in the 3 cases after the surgery. OCT showed that the FTMH remained closed at the last follow-up examination in 2 patients and vision improved to 20/20 and 20/25. In the other patient, the hole was closed temporarily after surgery, but was reopened at 6 months. The vision had improved to 20/60 until the hole was reopened, and it was 20/100 at the final follow-up examination. Lessons: Although only 3 patients were examined, the inverted ILM-flap technique may be an effective and safe method to close an FTMH in patients with MacTel type 2. However, the surgery cannot prevent the reopening of the hole when the retinal atrophy progresses.

Use of OCT Angiography to Diagnose and Manage Atypical Presentations of Macular Telangiectasia Type 2

“…This surgical intervention requires several days of face-down positioning post-operatively. The few studies that have evaluated the effectiveness of PPV have noted no significant differences in visual acuity between MacTel eyes with macular holes treated surgically compared to those that were medically managed without surgery [ 25 ], lower rates of macular hole closure with PPV in MacTel eyes compared to idiopathic macular holes without MacTel also treated surgically [ 26 ], and overall limited reports of successful macular hole closure in a number of eyes with MacTel [ 27 , 28 ]. Hence, surgical intervention of concomitant macular holes with MacTel may not be the preferred treatment for all patients.…”