Tacrolimus induced optic neuropathy in post-lung transplant patients: A series of 3 patients.

PURPOSE: Tacrolimus is a commonly used immunosuppressant medication after lung transplantation. In rare cases, tacrolimus causes a medication-induced optic neuropathy (TON) that can lead to significant vision loss. OBSERVATIONS: In this series, we describe three cases of TON, 1-10 years after medication use. Two patients were young (22yr and 33yr) females with cystic fibrosis. The last case was a 65yr male with idiopathic pulmonary fibrosis. In 2/3 cases tacrolimus serum levels were normal. Visual acuity ranged from 20/20 to 20/300, and vision loss occurred acutely to sub-acutely, over a span of 2-3 months. CONCLUSIONS AND IMPORTANCE: As presented here, TON can be highly variable. MRI findings are often non-specific, from normal brain findings to extensive white matter changes. There remains an unclear association with graft-versus-host disease and reduced kidney function. Visual findings are often subtle, including color vision aberration and peripheral visual field deficits, both of which usually require an ophthalmologic evaluation. When diagnosed in a timely fashion, TON is at least partially reversible in up to half of all cases. While rare, the cases described here support post-lung transplant ophthalmologic evaluation in those taking high-risk medications

Reticular Pseudodrusen in Early Age-Related Macular Degeneration Are Associated With Choroidal Thinning

PURPOSE. To compare choroidal thickness (CT) measurements in early AMD between patients with and without reticular pseudodrusen (RPD) using spectral-domain optical coherence tomography (SD-OCT). METHODS. This cross-sectional study examined 84 age-and sex-matched AMD patients (40 RPD [63 eyes], 44 non-RPD [75 eyes]). Fundus photographs and scanning laser ophthalmoscopy images were graded to identify RPD and non-RPD groups by three retinal specialists (MO, SY, SB) who were masked to corresponding SD-OCTs. CT at the fovea and 2400 to 3000 lm superior and inferior to the fovea was measured on SD-OCT by a grader (AG) and reviewed by a retinal specialist (SB). Only images with a clear posterior choroidal margin were analyzed (six eyes excluded due to poor image quality), and enhanced depth imaging SD-OCT was used when available (20 of 138 eyes). Greatest retinal thickness (RT) on horizontal foveal SD-OCT was also recorded. RESULTS. Mean CTs in the superior, foveal, and inferior macula in RPD (191.3 lm 6 57.9 SD, 176.3 lm 6 60.5 SD, 179.7 lm 6 56.24 SD) were significantly less than that of non-RPD (228.0 lm 6 66.1 SD, 216.5 lm 6 70.3 SD, 224.4 lm 6 71.9 SD; P ¼ 0.0010, P ¼ 0.0005, P ¼ 0.0001, respectively), as was greatest RT (P ¼ 0.0301). CONCLUSIONS. CT was thinner throughout the macula in the RPD group as compared with the non-RPD group. The current analysis supports an association between RPD and a thinned choroidal layer and is consistent with a choroidal etiology of RPD. CT may be integral to understanding RPD, and may be helpful in stratifying AMD progression risk. Keywords: reticular pseudodrusen, spectral-domain optical coherence tomography, choroidal layer, early age-related macular degeneration, retinal ischemia A ge-related macular degeneration is the leading cause of vision loss in developing and developed countries, 1 and accounts for more than 54% of all blindness in the United States. 2 Age-related macular degeneration is a disease characterized by extracellular material, collectively described as drusen, which accumulates under the RPE. Reticular pseudodrusen (RPD) are found in the fundus of some patients with AMD and were initially characterized as a peculiar yellow pattern occurring in the outer macula best visible under blue light in photographs of patients with AMD. 3 RPD have been associated with a higher likelihood of developing late AMD in the forms of choroidal neovascularization (CNV) 4-9 and geographic atrophy (GA). 10 However, there is no clear agreement about the prevalence of RPD, the relationship between RPD and AMD, 11 or the underlying etiology of RPD. Theories proposed for the etiology of RPD include abnormal choroidal perfusion, subretinal drusenoid deposits (SDD), or a combination of both. RPD have been associated with choroidal vascular abnormalities. A histopathologic study of one RPD eye found that there was a loss of the inner and middle layers of the choroid, subsequently leading to fibrous replacement of choroidal stroma. In normal eyes, the choroidal layer has been found to be thickest in the subfoveal region, where visual acuity and the concentration of cone photoreceptors are highest, with progressive thinning outward in both the horizontal and vertical axes

Is Reticular Macular Disease a Choriocapillaris Perfusion Problem?

The etiology of reticular macular disease (RMD), a sub-phenotype of age-related macular degeneration (AMD), is controversial and has not been clarified. RMD is suspected to be a multifactorial, complex disease with genetic, environmental, and systemic factors playing an important role in its origin. Findings from combinations of different imaging modalities suggest that the pattern that characterizes this condition is associated with an alteration of the choriocapillaris blood flow. If the choroid is indeed affected in RMD, the possible linkage with inflammatory or other systemic diseases could be better supported

Intraretinal Correlates of Reticular Pseudodrusen Revealed by Autofluorescence and En Face OCT.

Purpose We sought to determine whether information revealed from the reflectance, autofluorescence, and absorption properties of RPE cells situated posterior to reticular pseudodrusen (RPD) could provide insight into the origins and structure of RPD. Methods RPD were studied qualitatively by near-infrared fundus autofluorescence (NIR-AF), short-wavelength fundus autofluorescence (SW-AF), and infrared reflectance (IR-R) images, and the presentation was compared to horizontal and en face spectral domain optical coherence tomographic (SD-OCT) images. Images were acquired from 23 patients (39 eyes) diagnosed with RPD (mean age 80.7 ± 7.1 [SD]; 16 female; 4 Hispanics, 19 non-Hispanic whites). Results In SW-AF, NIR-AF, and IR-R images, fundus RPD were recognized as interlacing networks of small scale variations in IR-R and fluorescence (SW-AF, NIR-AF) intensities. Darkened foci of RPD colocalized in SW-AF and NIR-AF images, and in SD-OCT images corresponded to disturbances of the interdigitation (IZ) and ellipsoid (EZ) zones and to more pronounced hyperreflective lesions traversing photoreceptor-attributable bands in SD-OCT images. Qualitative assessment of the outer nuclear layer (ONL) revealed thinning as RPD extended radially from the outer to inner retina. In en face OCT, hyperreflective areas in the EZ band correlated topographically with hyporeflective foci at the level of the RPE. Conclusions The hyperreflective lesions corresponding to RPD in SD-OCT scans are likely indicative of degenerating photoreceptor cells. The darkened foci at positions of RPD in NIR-AF and en face OCT images indicate changes in the RPE monolayer with the reduced NIR-AF and en face OCT signal suggesting a reduction in melanin that could be accounted for by RPE thinning

Is Reticular Macular Disease a Choriocapillaris Perfusion Problem?

The etiology of reticular macular disease (RMD), a sub-phenotype of age-related macular degeneration (AMD), is controversial and has not been clarified. RMD is suspected to be a multifactorial, complex disease with genetic, environmental, and systemic factors playing an important role in its origin. Findings from combinations of different imaging modalities suggest that the pattern that characterizes this condition is associated with an alteration of the choriocapillaris blood flow. If the choroid is indeed affected in RMD, the possible linkage with inflammatory or other systemic diseases could be better supported

The controversial drive for intervention with ophthalmologic screening for Candida bloodstream infections

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Quantitative Fundus Autofluorescence and Optical Coherence Tomography in ABCA4 Carriers

PURPOSE. To assess whether carriers of ABCA4 mutations have increased RPE lipofuscin levels based on quantitative fundus autofluorescence (qAF) and whether spectral-domain optical coherence tomography (SD-OCT) reveals structural abnormalities in this cohort. METHODS. Seventy-five individuals who are heterozygous for ABCA4 mutations (mean age, 47.3 years; range, 9-82 years) were recruited as family members of affected patients from 46 unrelated families. For comparison, 57 affected family members with biallelic ABCA4 mutations (mean age, 23.4 years; range, 6-67 years) and two noncarrier siblings were also enrolled. Autofluorescence images (308, 488-nm excitation) were acquired with a confocal scanning laser ophthalmoscope equipped with an internal fluorescent reference. The gray levels (GLs) of each image were calibrated to the reference, zero GL, magnification, and normative optical media density to yield qAF. Horizontal SD-OCT scans through the fovea were obtained and the thicknesses of the outer retinal layers were measured. RESULTS. In 60 of 65 carriers of ABCA4 mutations (age range, 9-60), qAF levels were within normal limits (95% confidence level) observed for healthy noncarrier subjects, while qAF levels of affected family members were significantly increased. Perifoveal fleck-like abnormalities were observed in fundus AF images in four carriers, and corresponding changes were detected in the outer retinal layers in SD-OCT scans. Thicknesses of the outer retinal layers were within the normal range. CONCLUSIONS. With few exceptions, individuals heterozygous for ABCA4 mutations and between the ages of 9 and 60 years do not present with elevated qAF. In a small number of carriers, perifoveal fleck-like changes were visible

Acute Solar Retinopathy and the Autosomal Dominant Compelling Helio-Ophthalmic Outburst Syndrome

The photic sneeze reflex, also known as autosomal dominant compelling helio-ophthalmic outburst (ACHOO)syndrome, is found in approximately 1 in every 4 individuals.We report the case of a young man who elicited his own photic sneeze reflex in order to clear the congestive symptoms of an upper respiratory tract infection, resulting in acute solar retinopathy

Quantitative Comparison of Drusen Segmented on SD-OCT versus Drusen Delineated on Color Fundus Photographs

Drusen were segmented and measured by using SD-OCT and color fundus photography. Differences between the two modalities were categorized and quantified