To determine the degree of concordance for progression rate, size of atrophy, and visual acuity in patients with bilateral geographic atrophy (GA) due to age-related macular degeneration (AMD).Analysis was performed in 156 eyes of 78 patients with bilateral GA. Best corrected visual acuity was determined with ETDRS charts. GA was quantified in digital fundus autofluorescence images (excitation, 488 nm; emission, >500 nm) by semiautomated imaging analysis. A linear, two-level, random-effects model was used to assess the natural course of disease. The concordance correlation coefficient (CCC) was calculated to assess the degree of agreement between disease characteristics of the left and right eyes. Bland-Altman plots were applied to compare measurements in the eyes.CCC between the eyes was 0.310 (95% CI, 0.097-0.495) for visual acuity, 0.706 (95% CI, 0.575-0.801) for GA size, and 0.756 (95% CI, 0.644-0.837) for GA progression rate. Although Bland-Altman plots revealed high concordance for the progression rate, there was considerable discrepancy between both eyes for GA size. [corrected]GA progression in bilateral atrophic AMD is a symmetrical process; however, GA size may differ substantially between the eyes. High concordance in intraindividual disease progression in the presence of a high degree of interindividual variability indicates an influence by genetic and/or environmental factors rather than nonspecific ageing changes. The relatively small concordance of GA size in this cohort may indicate asymmetric evolution of the disease in affected individuals. The results may be useful in the design of future clinical trials designed to slow the rate of GA progression (Clinical Trials.gov number, NCT00393692).
Intraoperative optical coherence tomography (iOCT) is used extensively in complex anterior segment surgery, providing increased precision and thereby more safety and improved clinical outcomes. In lamellar keratoplasty, the successful application of iOCT has already been described [1]. For example, the iOCT can be used for defining the cutting depth of deep anterior lamellar keratoplasty (DALK) or for visualization of potential fluid in the interface in Descemet stripping automated endothelial keratoplasty (DSAEK) [2], [3], [4]. Moreover, for the correct corneal graft positioning in Descemet membrane endothelial keratoplasty (DMEK), a marker was previously required. The iOCT allows recognition of the corneal graft orientation and further observation of the unfolding process of the graft without the need for marking [1], [5].
Abstract Purpose Spontaneous corneal perforation is a rare complication of systemic diseases with ocular involvement such as Sjögren's syndrome, rheumatoid arthritis, graft‐versus‐host disease or ocular rosacea. There are few cases of spontaneous perforations in adult patients described in the literature associated with keratoconus (KC) or pellucid marginal degeneration (PMD). This case describes a spontaneous corneal perforation in a young patient with vernal keratoconjunctivitis. Methods Case report. Results A 14‐year‐old male patient presented at the Department of Ophthalmology accompanied by his parents due to one day existing redness and hazy vision of the left eye (OS). Additionally, he noticed a sudden “water outflow from the left eye.” The patient's medical history includes vernal keratoconjunctivitis, systemic prediabetes, obesity and steatohepatitis. Slit‐lamp examination of the left eye revealed a spontaneous corneal perforation of 1.4 × 1.8 mm inferonasal with iris tamponade and a surrounding corneal melting of 4 mm. The anterior chamber had been totally flattened. The right eye was diagnosed by slit‐lamp examination with central corneal thinning and inferior peripheral vascularization. The corneal perforation was primarily treated with histoacrylic tissue glue. Antibiotic treatment with Ceftazidime and Moxifloxacin eye drops alternating every hour has been established. After one week, a progressive corneal melting in the area of the primary corneal perforation was observed. Therefore, a patch “keratoplasty à chaud” had to be performed. The postoperative course was devoid of complications. The patient was initially treated with Pred forte eye drops 4 times daily. An optical penetrating keratoplasty is planned. Conclusions Spontaneous corneal perforation is an extremely rare but severe complication of vernal keratoconjunctivitis, which can also occur in young patients. Ophthalmologists should be aware of this rare complication and inform and treat their patients accordingly.
Human organoids recapitulating the cell-type diversity and function of their target organ are valuable for basic and translational research. We developed light-sensitive human retinal organoids with multiple nuclear and synaptic layers and functional synapses. We sequenced the RNA of 285,441 single cells from these organoids at seven developmental time points and from the periphery, fovea, pigment epithelium and choroid of light-responsive adult human retinas, and performed histochemistry. Cell types in organoids matured in vitro to a stable "developed" state at a rate similar to human retina development in vivo. Transcriptomes of organoid cell types converged toward the transcriptomes of adult peripheral retinal cell types. Expression of disease-associated genes was cell-type-specific in adult retina, and cell-type specificity was retained in organoids. We implicate unexpected cell types in diseases such as macular degeneration. This resource identifies cellular targets for studying disease mechanisms in organoids and for targeted repair in human retinas.
Abstract Purpose: To assess disease progression qualitatively and quantitatively in RPGR ‐associated X‐linked retinitis pigmentosa ( RPGR‐ XLRP). Methods: PubMed was used to identify relevant studies (01/2000–09/2021). Search terms included RP, XLRP, history, progress, disease state, health state, and burden. Included studies focused on RPGR ‐XLRP (ie, results specific to RPGR or RP studies with ≥80% of patients with RPGR variants). Endpoints of interest included changes in visual acuity (VA), visual field (VF), and ellipsoid zone width (EZW), progression to legal blindness, and patient‐reported outcomes (PROs). Results: Of 1325 studies captured by search criteria (including those examining XLRP [ n = 17], RP [127], and both XLRP and RP [6]), 11 met inclusion criteria for assessment of RPGR ‐XLRP disease progression. In those with RPGR ‐XLRP, data suggest progressive decline in VA, VF, and EZW. Annual changes in VA were equivocal, with two studies reporting positive or minimal change in best‐corrected VA (BCVA; +2%, −0.07%) and two studies reporting VA decline (−3.5 to −5.3%, −8.2%). Annual declines in VF, measured via Goldmann or Humphrey perimetry, were observed across studies (range, −4.5, −9.0%; n = 4 studies). EZW, measured via Heidelberg Spectralis OCT, became constricted over time ( n = 5 studies); 2 studies showed an 8%–9% annual exponential rate of decline. In two studies, patients reached VA‐based legal blindness (BCVA <20/400 in the better seeing eye or <6/60) by median ages of 48 and 51.3 years; in another study, 18% reached blindness (BCVA <20/400) by age 40. PRO data in this patient population were limited. Conclusions: Published evidence shows that those with RPGR ‐XLRP experience significant progressive decline in VA, VF, and EZW, eventually resulting in blindness. There is a need for more standardized longitudinal data and expanded collection of PROs to better assess visual decline in RPGR ‐XLRP.
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