Retinal pigment epithelial detachments and tears, and progressive retinal degeneration in light chain deposition disease

Autor: L Spielberg, Anita Leys, John R. Heckenlively
Rok vydání: 2013
Předmět:
Indocyanine Green
Male
Retinal degeneration
medicine.medical_specialty
genetic structures
Paraproteinemias
Visual Acuity
Retinal Pigment Epithelium
Fundus (eye)
Retina
Light chain deposition disease
Cellular and Molecular Neuroscience
Ophthalmology
Humans
Medicine
Metamorphopsia
Fluorescein Angiography
Coloring Agents
Retinal pigment epithelium
medicine.diagnostic_test
Choroid
business.industry
Retinal Degeneration
Retinal Detachment
Fundus photography
Retinal detachment
Anatomy
Middle Aged
Clinical Science
Retinal Perforations
medicine.disease
Fluorescein angiography
eye diseases
Sensory Systems
medicine.anatomical_structure
Disease Progression
Degeneration
Visual Field Tests
Female
Immunoglobulin Light Chains
sense organs
Visual Fields
medicine.symptom
business
Tomography
Optical Coherence
Follow-Up Studies
Zdroj: The British Journal of Ophthalmology
ISSN: 1468-2079
0007-1161
DOI: 10.1136/bjophthalmol-2012-302851
Popis: Background/purpose Light-chain deposition disease (LCDD) is a rare condition characterised by deposition of monoclonal immunoglobulin light chains (LCs) in tissues, resulting in varying degrees of organ dysfunction. This study reports the characteristic clinical ocular findings seen in advanced LCDD upon development of ocular fundus changes. This is the first report to describe this entity in vivo in a series of patients. Methods A case series of ocular fundus changes in three patients with kidney biopsy-proven LCDD. All patients underwent best corrected visual acuity (BCVA) exam, perimetry, colour fundus photography and fluorescein angiography; two patients underwent indocyanine green angiography, optical coherence tomography, ultrasound and electroretinography; and one patient underwent fundus autofluorescence. Results Three patients, 53–60 years old at initial presentation, were studied. All three presented with night blindness, poor dark adaptation, metamorphopsia and visual loss. Examination revealed serous and serohaemorrhagic detachments, multiple retinal pigment epithelial (RPE) tears, diffuse RPE degeneration and progressive fibrotic changes. Neither choroidal neovascularisation nor other vascular abnormalities were present. Final best corrected visual acuity (BCVA) ranged from 20/40 to 20/300. Conclusions Progressive LC deposition in the fundus seems to damage RPE pump function with flow disturbance between choroid and retina. This pathogenesis can explain the evolution to RPE detachments and subsequent rips and progressive retinal malfunction.
Databáze: OpenAIRE
Externí odkaz: