Retinal pigment epithelial detachments and tears, and progressive retinal degeneration in light chain deposition disease
Case 2 concerns a 60-year-old man who presented in 2003 with a 2-year history of poor vision in the left eye and a 5-month history of visual loss in the right eye. He had a history of multiple myeloma diagnosed 16 years earlier, which was treated with chemotherapy. Six years before initial presentation to the medical retina department, the patient had developed end-stage renal disease. Kidney biopsy showed LC deposits. Renal failure was treated with haemodialysis. Arterial hypertension was medically controlled. The patient had neither diabetes mellitus nor primary renal disease.\nA general ophthalmologist's report, dating from 2001, 2 years before presentation to our department, indicated a peripheral RPE detachment and retinal detachment in the left eye and no obvious changes in the right eye. At initial presentation in our department, BCVA was 20/200 and 20/60 with bilateral metamorphopsia. The left eye had annular fibrosis in the mid-periphery (A). Fluorescein angiography of the right eye (B) showed a macular neurosensory detachment and a recent and two older tears of the RPE, as well as extensive supramacular and inframacular haemorrhages in the right eye. There was no evidence of choroidal neovascularisation (CNV).\nOne month later, there was a confluence of haemorrhages in the right eye (C,D). Nine months after baseline, fibrotic changes were also evident in the right eye (E). Progressive devastation of the posterior pole was seen at 13 months after baseline. BCVA was 20/300 OD and 20/70 OS. The patient succumbed to systemic complications related to renal failure shortly thereafter. No ocular pathological study could be performed.