Role of acetazolamide in tuberous sclerosis related macular edema.

Author: Talal Asad (United Kingdom)

Co-authors: Aamir S Pasha, Khine Noe, S Ahmed Aftab

Purpose

To report visual and anatomic outcome of tuberous sclerosis patient with macular oedema treated with acetazolamide.

Setting/Venue

An 27 year female was referred to eye clinic by optician with 2 years history of blurred vision for maculpathy with background of tuberous sclerosis.Patient was diagnosed with tuberous sclerosis by paediatricians when she was 5 years of age.

Methods

Study design: Case report Summary: A 27 year female was referred to eye clinic by optician with 2 years history of blurred vision for maculpathy with background of tuberous sclerosis. Patient was diagnosed with tuberous sclerosis by paediatricians when she was 5 years of age. On presentation she had visual acuity of 6/15 and 6/18 in right and left eye respectively. She had unremarkable anterior segments, mutiple retinal astrocytic hamartomas. Macular edema was detected bilaterally. A trial of topical NSAID (non steroidal anti inflammatory drug) g.Bromefenac 0.9mg/ml 1 drop twice per day was commenced for both eyes, with out any success.Topical carbonic anhydrase inhibitor g.brinzolamide 10mg/ml 1 drop twice per day for both eyes made minimal improvement transiently did not resolve macular edema completely. Patient was switched to topical NSAID g.Bromefenac 1 drop BD again which again remained unsuccessful in treating macular edema As a next step, funding request was submitted for intravitreal steroid to treat macular edema , but funding request was not approved. Oral Acetazolamide sustained release formulation 250 gram twice per day was commenced and patient was asked to continue topical NSAID bromefenac 1 drop twice per day as well.

Results

By adding oral acetazolamide sustained release formulation 500mg per day, her macular edema resolved completely for the first time and that also within 4 weeks. Central sub foveal thickness on OCT macula reduced from 552 and 483 micrometers in the right and left eye respectively to 272 micrometres in the each eye. Best corrected visual acuity improved from 6/18 and 6/24 in the right eye and left eye respectively to 6/9 and 6/12 in right and left eye respectively, which was better than her visual acuity at initial presentation. The effect remained sustained for more than 18 months . Her visual acuity at 18th month follow up was with central subfoveal thickness 271 and 173 micrometres in the right and left eye respectively

Conlusions

In literature, there is mention of intravitreal bevacizumab and intravitreal steroid for the management of macular edema due to tuberous sclerosis complex . Use of oral Acetazolamide has not been reported to our knowledge. Acetazolamide can be a safe , cost effective alternative in tuberous sclerosis patients with macular edema.

Financial Disclosure

No known financial relation of any of the authors