A case of macular telangiectasia type 2 with bilateral macular holes: Imaging features and surgical management

A macular hole (MH) secondary to macular telangiectasia (MacTel) type 2 is generally considered a poor surgical candidate when internal limiting membrane (ILM) peeling is performed. Due to the scarcity of studies on MH in MacTel type 2 patients, the number of published studies exploring the surgical outcomes of these patients is limited.

In this case report, we report a case with MacTel type 2 with bilateral MHs and had a good visual prognosis after surgical treatments.

A 66-year-old woman was referred to our hospital for macular abnormalities in the right eye. The best-corrected visual acuity (BCVA) was 20/32 in the right eye and 20/16 in the left eye at the initial examination. Ophthalmic examination revealed decreased retinal transparency temporal to the fovea in both eyes, cystoid spaces at the macula of the right eye by optical coherence tomography, telangiectasia temporal to the fovea, and fluorescent leakage temporal to the fovea by fluorescein angiography in both eyes and the patient was diagnosed with MacTel type 2.

Since there was no tendency toward vision loss, the patient was followed up without any treatment. Four years later, the BCVA of the right eye had decreased to 20/50, and outer retinal layer atrophy at the macula had progressed, so the patient underwent cataract surgery, pars plana vitrectomy (PPV), and fluid-air exchange. Seven months after the initial surgery, an MH was formed in the right eye. Second surgery including PPV, ILM peeling, and sulfur hexafluoride gas tamponade were performed.

Postoperatively, the MH was closed, and the BCVA improved to 20/32 at 1 year, and 20/20 at 12 years after the surgery, respectively. Twelve years after the initial visit, an MH appeared in the left eye, and the BCVA decreased to 20/50, so cataract surgery, PPV with inverted ILM-flap technique, and sulfur hexafluoride gas tamponade were performed in the left eye. Postoperatively, the MH was closed with no recurrence for longer than 2 years after surgery, and the BCVA improved to 20/16. Microperimetry performed after the surgery showed decreased retinal sensitivity consistent with areas of retinal atrophy in both eyes.

We experienced a case of bilateral MHs associated with MacTel type 2. The right eye underwent PPV with ILM peeling and the left eye underwent PPV with an inverted ILM flap technique, both eyes had good visual prognosis. Imaging modalities and microperimetry can provide valuable information on the associated anatomical and functional changes.