Korean J Ophthalmol.  2014 Apr;28(2):189-191. 10.3341/kjo.2014.28.2.189.

Acetazolamide for Cystoid Macular Oedema in Bietti Crystalline Retinal Dystrophy

• Broadhead
• Chang AA

• KMID: 1792116
• DOI: http://doi.org/10.3341/kjo.2014.28.2.189

Abstract

Bietti crystalline retinal dystrophy is a rare, inherited disorder whose hallmark is the presence of retinal crystal deposits associated with later chorioretinal degeneration. This condition may rarely be complicated by the development of cystoid macular oedema leading to rapid visual decline. Currently, treatment options for this complication of Bietti dystrophy are limited and the visual prognosis is poor. Here, we present a case of cystoid macular oedema associated with Bietti dystrophy that was successfully diagnosed using multimodal imaging techniques including optical coherence tomography and fluorescein angiography. These modalities confirmed the diagnosis of macular oedema and excluded other possible causes of oedema such as choroidal neovascularisation. In this patient, cystoid macular oedema was resolved with oral acetazolamide therapy, a treatment that has not been previously reported in this context. Acetazolamide treatment resulted in oedema resolution and improvement in visual function, and can be considered a therapeutic option for other patients with Bietti dystrophy who develop cystoid macular oedema.

Keyword

Acetazolamide; Bietti crystalline dystrophy; Macular edema; Optical coherence tomography

MeSH Terms

Acetazolamide/*administration & dosage
Administration, Oral
Adult
Corneal Dystrophies, Hereditary/*drug therapy/pathology
Diuretics/*administration & dosage
Humans
Macular Edema/*drug therapy/pathology
Male
Retinal Diseases/*drug therapy/pathology
Tomography, Optical Coherence
Treatment Outcome
Acetazolamide
Diuretics

Figure

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  Fig. 1 Images from the patient demonstrating crystalline retinopathy. (A) Fundus photograph of the right eye, (B) fundus photograph of the left eye, (C) early-phase fundus fluorescein angiogram (FFA) of the right eye, (D) early-phase FFA of the left eye, (E) late-phase FFA of the right eye, (F) late-phase FFA of the left eye, (G) baseline time-domain optical coherence tomography (TD-OCT) image of the right eye, (H) baseline TD-OCT image of the left eye, (I) 1-year post-treatment TD-OCT image of the right eye, and (J) 1-year post-treatment TD-OCT image of the left eye.

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