Multimodal Imaging of the Retina and Choroid in Systemic Amyloidosis

Roybal, C. Nathaniel; Sanfilippo, Christian J.; Nazari, Hossein; Law, Janice C.; Bhaleeya, Swetangi; Ming, Gemmy Cheung Chui; Rao, Narsing A.; Kiss, Szilárd; Sadda, Srinivas R.; Sarraf, David

doi:10.1097/icb.0000000000000215

Cited by 20 publications

(20 citation statements)

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“…The subretinal deposits observed in this case are different from those found in the membranoproliferative glomerulonephritis [13,14]. The pachychoroid feature with a widened choriocapillaris was first outlined by Roybal [11] and may be secondary to light chain deposition as suggested in several postmortem histological studies [15,16]. In particular in the latter study, electron microscopic and immunohistochemistry from ocular specimens of a patient with kappa AL amyloidosis revealed kappa light chain deposits within Bruch membrane and in the innermost part of the choroid associated with choriocapillaris obstructions and macular exudative retinal detachments.…”

Section: Discussionmentioning

confidence: 52%

“…The incidence of this clinical presentation is probably underestimated as it is largely overlooked in these patients. To our knowledge, among the published cases of AL amyloidosis with chorioretinal disease [10][11][12], our case is one of the first to document the rapid progression of fundus alterations and their stabilization after disease remission. Elementary lesions in our case include pachychoroid, large subretinal detachments, which resolved after chemotherapy and subretinal deposits that are hyperautofluorescent leading to masking on angiography.…”

Section: Discussionmentioning

confidence: 63%

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Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

2020

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Background: To report an unusual case of light-chain (AL) amyloidosis with progressive bilateral chorioretinal abnormalities documented with short-wavelength autofluorescence, SD-OCT, fluorescein and indocyanine green angiography. Case presentation: Case report of a forty-three-year-old male patient with kappa AL amyloidosis. The patient presented with rapidly progressing pigmented and hyperautofluorescent drusenoid deposits in both eyes, associated with central serous retinal detachments, a pachychoroid and choriocapillaris enlargement. The general assessment revealed a renal failure symptomatic of a nephrotic syndrome, associated with proteinuria composed mainly of free kappa light chains. A kidney biopsy confirmed the diagnosis of kappa AL amyloidosis. Chemotherapy was quickly started. During remission, the extension of drusenoid deposits on the fundus was stopped and a disappearance of the subretinal fluid on SD-OCT was observed. Conclusions: AL amyloidosis is an insidious and potentially fatal condition. This case is one of the first to document the rapid progression of fundus alterations and their stabilization after disease remission. Identifying these specific fundus abnormalities is essential to avoid diagnosis wandering and therapeutic delay.

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Section: Discussionmentioning

confidence: 52%

Section: Discussionmentioning

confidence: 63%

Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

2020

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“…The OCTA images of the eyes of our patient with severe systemic amyloydosis due to CAPS showed not only a reduction of the retinal capillary density at the inner retinal layer but also the loss of choriocapillaris blood flow at the hyporeflective choriocapillaris band in the cross sectional OCT. Roybal et al 6 conducted a retrospective analysis of four patients with amyloidosis-induced chorioretinopathy, and they reported a thicker hyporeflective choriocapillaris band in the OCT images. This band is similar to the hyporeflective band in the SS-OCT images in our case.…”

Section: Discussionmentioning

confidence: 99%

Retinal and choroidal circulation determined by optical coherence tomography angiography in patient with amyloidosis

et al. 2019

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A 43-year-old woman who was diagnosed with the cryopyrine-associated periodic syndrome (CAPS) with severe renal failure and heart failure due to amyloid accumulation was examined by swept source optical cohernce tomography (OCT) (SS-OCT; DRI-OCT, Topcon, Tokyo, Japan) and optical coherence tomography angiography (OCTA) (RTVue XR Avanti, Optovue, Fremont, CA). Her best-corrected visual acuity was 20/40 OD and 20/25 OS. A hyporeflective band of about 100 µm thickness was seen just inferior to the retinal pigment epithelium in the cross-sectional SS-OCT images, but the deeper choroidal structures were clearly visible. In the OCTA images, the density of the retinal capillaries in the superficial and deep capillary plexus slabs were reduced, and no signals of the choroidal capillary slab was detected after removing the projection artefacts. The accumulation of amyloid can cause a reduction of both the retinal and choroidal capillary circulations although the circulation in the larger vessels are preserved.

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“…The specific combination of pachychoroid features and a widened choriocapillaris was first reported by Roybal et al in a case series of 4 patients with systemic amyloidosis. 7 Characteristic features of amyloid-associated chorioretinopathy on spectral domain OCT include a widened choriocapillaris band, choroidal infiltration and thinning of the outer nuclear layer, and these changes were associated with a decline in visual function. The authors surmise that amyloid infiltration and occlusion may disturb the RPE and photoreceptor function and eventually lead to outer retinal atrophy.…”

Section: Discussionmentioning

confidence: 99%

“…In a recent case of amyloidosis encountered by us, amyloidosis was associated with chorioretinopathy, and OCT showed a thick hyporeflective choriocapillaris band in the chorioretinal area. 6 , 7 To the best of our knowledge, there are no reports of long-term observation of amyloidosis patients with ocular manifestations in the literature to date. Herein, we present the first report of long-term follow-up of a case of amyloidosis-associated chorioretinopathy by imaging studies, including OCT.…”

Section: Introductionmentioning

confidence: 99%

Long-term follow-up of a case of amyloidosis-associated chorioretinopathy

Nagura

Inoue

Ching

et al. 2020

American Journal of Ophthalmology Case Reports

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Purpose To describe the findings of long-term follow-up of a case of amyloidosis-associated chorioretinopathy by multimodal imagings, including optical coherence tomography (OCT). Observations A 47-year-old woman who had been diagnosed as having systemic amyloidosis was found to have a best corrected visual acuity of 20/13 in both eyes at the age of 41, which subsequently decreased to 20/100 in the left eye and 20/20 in the right eye at age 47. Visual field examination demonstrated worsening of the central scotoma during the follow-up period. Funduscopic examination revealed bilateral white deposits along the choroidal vessels, which became more pronounced over time, along with diffuse pigmentary changes. The fluorescein angiography and indocyanine green angiography findings were consistent not only with atrophic lesions, but also with amyloid deposition (i.e., staining of the vessels). At the baseline, macula OCT revealed a thick hyporeflective band at the choriocapillaris, however, at the last follow-up, it revealed an absent ellipsoid zone, and bilateral progressive retinal pigment epithelium irregularities in both eyes. Conclusions Patients diagnosed as having amyloidosis-related chorioretinopathy may have maintained visual function at the first detection of retinal amyloid deposition, and a number of years may elapse before the patient manifests visual deterioration.

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Multimodal Imaging of the Retina and Choroid in Systemic Amyloidosis

Cited by 20 publications

References 12 publications

Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

Retinal and choroidal circulation determined by optical coherence tomography angiography in patient with amyloidosis

Long-term follow-up of a case of amyloidosis-associated chorioretinopathy

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