Retinal pigment epithelial detachments and tears, and progressive retinal degeneration in light chain deposition disease
Case 3 concerns a 53-year-old man with end-stage renal disease and biopsy-proven LCDD. The patient had neither diabetes mellitus nor primary renal disease. He presented to the department of ophthalmology with complaints of night blindness and dark adaptation difficulty. The ERG was highly abnormal bilaterally. BCVA was 20/30 and 20/25. The visual fields were mildly constricted bilaterally, with a large deficit in the superonasal sector of the right eye. Colour fundus photography and red-free photography of the posterior pole of the right eye showed lipid exudates and a serohaemorrhagic RPE detachment temporal to the macula. Fluorescein angiography showed pooling of fluorescein in the region of the RPE detachment. A small RPE tear was also noted in the right eye (). Fibrosis and deep, subretinal bleeding were present in the peripheral retina of the right eye. Despite the abnormal retinal function tests, the fundus of the left eye was normal upon examination.