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A. Leys, L. Spielberg, J. R. Heckenlively; Retinal Pigment Epithelial Detachments and Tears in Light Chain Deposition Disease. Invest. Ophthalmol. Vis. Sci. 2010;51(13):4768.
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To report the characteristic clinical ocular changes in patients with light-chain deposition disease (LCDD). These clinical changes correspond to the progressive light chain deposition in Bruch’s membrane and choroid, as described histopathologically by B.C. Daicker in 1995. This is the first report of this entity in-vivo.
A case series of ocular fundus changes in 3 patients with immunoproliferative syndromes, end-stage renal disease and biopsy-proven LCDD. Two patients had multiple myeloma and one had a benign immunoproliferative syndrome with kappa LCDD. All patients underwent a complete ophthalmological examination, best-corrected visual acuity (BCVA) exam, perimetry, color fundus photography, and fluorescein angiography (FA); two patients underwent indocyanine green angiography (ICGA), optical coherence tomography (OCT), ultrasound and electroretinography (ERG); and one patient underwent fundus autofluorescence (AF).
One patient (case #1), aged 53, presented with night blindness and poor dark adaptation; BCVA was 20/30 and 20/25. Funduscopy and FA showed multiple serous and sero-hemorrhagic retinal pigment epithelial (RPE) detachments, a small RPE tear and the ERG was very abnormal OU. Two patients (case #2, aged 64 and case #3, aged 60) presented with metamorphopsia and visual loss. Initially, serous RPE detachments were observed. Later in their respective 3- and 11-year follow-ups, sero-hemorrhagic detachments, RPE tears, progressive fibrotic changes and diffuse RPE degeneration were noted. ICGA showed an unusually dark choroid in areas with RPE detachment. Neither choroidal neovascularization (CNV) nor other choroidal or retinal vascular abnormalities were present. The ERG of case #3 deteriorated to nearly flat; at that stage, AF showed numerous large very dark areas and diffuse RPE abnormalities. Final BCVA was 20/300 and 20/70 (case #2) and 20/64- and 20/40 (case #3).
Progressive light-chain deposition in the choroid and in Bruch’s membrane leads to loss of the RPE pump function. This causes a disturbance in the flow between choroid and retina, leading to multiple serous and serohemorrhagic RPE detachments and tears. There is neither obvious inflammation nor CNV. However, there is a progressive malfunction of the retina, as indicated by poor vision, night blindness and profound deterioration of the ERG.
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