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Rapid resolution of retinoschisis with acetazolamide



To report the results of an acetazolamide (Diamox®) treatment regimen in a genetically confirmed case of X-linked juvenile retinoschisis (XLRS).


A patient with XLRS was prescribed acetazolamide (Diamox®) at a dose of 500 mg/day, then discontinued the treatment due to non-compliance for 4 days, and finally resumed the course of treatment. Best-corrected visual acuity, retinal structure, and function were monitored with autofluorescence, spectral domain-optical coherence tomography (SD-OCT), adaptive optics scanning laser ophthalmoscopy (AOSLO), and full-field electroretinogram (ERG). Full-field ERG was performed using DTL recording electrodes and Ganzfeld stimulation according to ISCEV standards.


Serial monitoring of the cysts by SD-OCT revealed a strong association between the effects of acetazolamide administration and the size of the schisis. A reduction in foveal cyst size was significant in as rapid as 6 days after acetazolamide initiation. AOSLO data revealed that the resolution of cone cell images improves as the foveal schisis decreases in size.


Efficacy of acetazolamide in patients with XLRS can be apparent in as rapid as a week of therapy. AOSLO can be a good method to evaluate the cone cells after acetazolamide treatment in the early stages of XLRS.

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This study was supported, in part, by the NIH Minority Access to Research Careers Grant T34 GM008718. Imaging facilities are supported by P30EY019007 from Core Support for Vision Research, NEI; C026448 from New York Stem Cell Science; the Macula Foundation Inc; and unrestricted funds from Research to Prevent Blindness. S. H. T. is a member of the RD-CURE Consortium and is supported by the Tistou and Charlotte Kerstan Foundation, NIH R01EY018213, the Research to Prevent Blindness Physician-Scientist Award, the Schneeweiss Stem Cell Fund, New York State (N09G-302 and N13G-275), the Foundation Fighting Blindness New York Regional Research Center Grant (C-NY05-0705-0312), the Joel Hoffman Fund, the Charles Culpeper Scholarship, the Laszlo Bito and Olivia Carino Foundation, Justin A. Manus, Kobi and Nancy Karp, Professor Gertrude Rothschild Stem Cell Foundation, and the Gebroe Family Foundation.

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Correspondence to Stephen H. Tsang.

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Zhang, L., Reyes, R., Lee, W. et al. Rapid resolution of retinoschisis with acetazolamide. Doc Ophthalmol 131, 63–70 (2015). https://doi.org/10.1007/s10633-015-9496-8

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  • Retinoschisis
  • Acetazolamide
  • OCT