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Chen, John J. MD, PhD; Sohn, Elliott H. MD; Folk, James C. MD; Mahajan, Vinit B. MD, PhD; Kay, Christine N. MD; Boldt, H. Culver MD; Russell, Stephen R. MD

doi: 10.1097/IAE.0000000000000093
Original Study

Purpose: To evaluate whether carbonic anhydrase inhibitors reduce the macular thickness and/or cystic spaces in patients with macular telangiectasia (MacTel) Type 2.

Methods: Retrospective review of patients with nonproliferative cystoid changes associated with MacTel seen at the University of Iowa between 2009 and 2012. Carbonic anhydrase inhibitors were used in 8 patients with MacTel Type 2. Five patients with MacTel Type 2 were observed during this period. Initial and final visual acuities were documented. The presence of cystic spaces and the retinal thickness were measured with spectral-domain optical coherence tomography.

Results: Patients treated with oral carbonic anhydrase inhibitors showed significant reduction in both the cystoid cavities and central macular thickness when compared with the patients who were observed (−12.2 μm; P = 0.020). The reduction in retinal thickness was more pronounced in patients receiving acetazolamide (−20.13 μm; P = 0.007) compared with methazolamide (−6.25 μm; P = 0.177). There was no significant change in visual acuity in patients receiving carbonic anhydrase inhibitors. Five patients with MacTel Type 2 did not receive treatment and demonstrated no change in visual acuity, cystoid cavities, or central macular thickness.

Conclusion: Oral carbonic anhydrase inhibitors, particularly acetazolamide, may decrease macular cystic cavities and reduce central macular thickness but does not appear to improve visual acuity. These findings have yet to be confirmed with a prospective treatment trial.

Patients with macular telangiectasia Type 2 often present with cystic spaces and decreased vision. Currently, there is no proven treatment for this condition. The authors present evidence that treatment of patients with nonproliferative changes with oral carbonic anhydrase inhibitors can decrease the cystic spaces and macular thickness.

Department of Ophthalmology and Visual Science, University of Iowa, Iowa City, Iowa.

Reprint requests: Elliott H. Sohn, MD, Department of Ophthalmology and Visual Science, University of Iowa, 200 Hawkins Drive, Iowa City, IA 52242-1091; e-mail:

None of the authors have any financial/conflicting interests to disclose.

Dr. Folk is the Judith (Gardner) and Donald H. Beisner Professor of Vitreoretinal Diseases and Surgery. Dr. Boldt is the Marion and Frederick Fuerste Professor of Ophthalmology.

© 2014 by Ophthalmic Communications Society, Inc.