A 55yearold woman in the United States, with a history of interstitial cystitis diagnosed at age 33 years, was referred for pattern macular dystrophy. Her primary complaint was difficulty reading,… Click to show full abstract
A 55yearold woman in the United States, with a history of interstitial cystitis diagnosed at age 33 years, was referred for pattern macular dystrophy. Her primary complaint was difficulty reading, despite using bifocals. Her medications included hydroxyzine, amitriptyline and pentosan polysulfate (PPS). She had taken 300 mg of PPS daily for 21 years (cumulative dose, 2.3 kg). Visual acuity with correction was 6/7.5 and intraocular pressure was normal in both eyes. Fundus examination revealed bilateral perifoveal pigmentary deposits without haemorrhage or exudate. Spectral domain optical coherence tomography (SDOCT; SPECTRALIS HRA + OCT, Heidelberg Engineering), demonstrated irregular retinal pigment epithelium and small pigment epithelial detachments in the maculae of both eyes without cystoid macular oedema. Nearinfrared reflectance imaging and autofluorescence showed hyperreflective patches and speckled areas of hypoand hyperautofluorescence, respectively, in each eye (Box 1). Fluorescein angiogram demonstrated retinal pigment epithelial staining without leakage. Cessation of PPS and routine monitoring were recommended. Subsequently, the patient developed unilateral choroidal neovascularisation of the left eye (Box 2), which was treated with an intravitreal antivascular endothelial growth factor agent.
               
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