Background: To date, there are no studies associating the dried blood spot (DBS) levels of globotrioasylsphingosine (lysoGb3) with quantitative optical coherence tomography angiography (OCTA) parameters in Fabry disease (FD) patients. Here, we aimed to investigate the association between OCTA vessel density (VD), vessel length density (VLD) with DBS lysoGb3. Methods: A retrospective, single center analysis of all consecutive FD patients enrolled at the Department of Ophthalmology of the University Hospital of Zurich from December 1st, 2017 to September 9th, 2020. An association between VD and VLD detected by OCTA and lysoGb3 was investigated using a linear mixed model. Results: A total of 57 FD patients (23 male, 34 female; 109 eyes) were included. Forty-one patients suffered from the classic phenotype and 16 from the later-onset phenotype. LysoGb3 inversely correlated with VD and VLD in both the superficial (VD: p = 0.034; VLD: p = 0.02) and deep capillary plexus (VD: p = 0.017; VLD: p = 0.018) in the overall FD cohort. Conclusions: Our study shows an association between lysoGb3 and OCTA VD and VLD. This supports the hypothesis that quantitative OCTA parameters might be useful as diagnostic biomarkers for evaluating systemic involvement in FD, and possibly other diseases.
Background: During the first COVID-19 pandemic outbreak, a new triage concept had to be implemented for patients with retinal diseases having a scheduled appointment at the medical retina clinic. In this study, we aimed to assess patients’ confidence in this triage concept and patients’ satisfaction regarding the received treatment during the outbreak. Methods: This retrospective study included all patients with a diagnosed retinal disease, triaged into three priority groups based on their condition’s urgency during lockdown. After restrictions were eased, a subset of previously triaged patients was interviewed to assess their confidence in the triage and their satisfaction regarding the received treatment during the pandemic. Results: In total, 743 patients were triaged during the lockdown. Over 80% received an urgent appointment (priority 1). Among all priority 1 patients, over 84% attended their appointment and 77% received an intravitreal injection (IVI), while 7% cancelled their appointment due to COVID-19. In post-lockdown interviews of 254 patients, 90% trusted the emergency regimen and received treatment. Conclusions: Our triage seemed to be useful in optimizing access to treatment for patients with retinal diseases. An excellent rating of patients’ confidence in the triage and satisfaction regarding the received treatment during the first COVID-19 outbreak could be achieved.
Whether post injectional acute intraocular pressure (IOP) increase is associated with decreased peripapillary and macular perfusion is still under debate. Here, we investigated early changes in the choroidal and retinal blood flow using OCTA imaging in a cohort of patients undergoing anti-VEGF intravitreal injections (IVI) for macular edema following retinal vein occlusion and diabetic retinopathy. In this prospective single-center, observational study, the pre- and post-IVI changes in retinal perfusion were examined via assessment of vessel length density (VLD) and vessel density (VD) in deep and superficial capillary segmentations (DCP and SCP), foveal avascular zone (FAZ) in SCP, as well as flow signal deficits in the choriocapillaris segmentation. Mean IOP significantly changed over the study course (p = 0.000; ANOVA). Measurements at 5 min post-IVI (33.48 ± 10.84 mmHg) differed significantly from baseline (17.26 ± 2.41 mmHg, p = 0.000), while measurements from one day, one week, and one-month post-IVI did not (p = 0.907, p = 1.000 and p = 1.000 respectively). In comparison to baseline, no changes in OCTA parameters, including FAZ, VD, VLD, and FV, were detected 5 min post-IVI. No significant alterations in OCTA parameters were observed during study course. Increased IOP spikes were detected post-IVI; however, no potential permanent ischemic retinal damage was suspected.
Purpose To investigate a possible microvascular component of poppers maculopathy (PMP) using optical coherence tomography angiography (OCTA). Methods Twelve patients suffering from poppers maculopathy were included. Health records, optical coherence tomography (OCT), and OCTA data was gathered and compared to a healthy control group (HC). PMP lesion type was determined by manifestation in OCT. OCTA-based evaluation of retinal vascular plexus and choriocapillaris (CC) was executed. Vessel density (VD) and vessel length density (VLD) in superficial and deep capillary plexus (SCP, DCP), as well as flow deficits (FD), within the foveal avascular zone (FAZ) in CC were assessed. Results Median age of PMP patients was 40 (min 24; max 64) years, all male. Eleven patients presented with ellipsoid zone-type lesions; one patient showed a vitelliform-type lesion. No qualitative microvascular changes between PMP patients and HC were identified. Quantitative values for VD and VLD of SCP and DCP did not differ in between the two groups. The analysis of FDs in CC showed no deviation from PMP patients to HC. Conclusions No vascular anomalies in qualitative and quantitative analysis in OCTA were detected in PMP patients. The constitution of the CC within FAZ of PMP patients does not differ from HC when assessed as FD.
Background: To analyze long-term ophthalmic clinical and multimodal imaging findings of disseminated Mycobacterium (M.) chimaera infection after cardiothoracic surgery among the Swiss Cohort. Methods: Systemic and multimodal ophthalmic imaging and clinical findings including rate of recurrence were reviewed and correlated to a previously proposed classification system of choroidal lesions and classification of ocular disease. Main Outcomes Measures: long-term clinical and multimodal ocular imaging findings of M. chimaera. Results: Twelve patients suffering from systemic infection from M. chimaera were included. Mean age at the first ophthalmic examination was 59 years (range from 48 to 66 years). Mean duration of the follow-up was 22.63 ± 17.8 months. All patients presented with bilateral chorioretinal lesions at baseline; 5 patients had additional signs, including optic disc swelling (2), choroidal neovascularization (1), retinal neovascularization (1) and cilioretinal vascular occlusion (1). Four recurrence events after discontinuation or adjustment of the antibiotic treatment were observed. Progressive choroiditis was seen in 5 patients under treatment, 4 of them deceased. Conclusions: Expertise from ophthalmologists is not only relevant but also critical for the assessment of the adverse drug effect of antimycobacterial treatment along with monitoring therapeutic response and identifying recurrences.
Due to their non-specific diagnostic patterns of ocular infection, differential diagnosis between Mycobacterium (M.) chimaera and tuberculosis can be challenging. In both disorders, ocular manifestation can be the first sign of a systemic infection, and a delayed diagnosis might reduce the response to treatment leading to negative outcomes. Thus, it becomes imperative to distinguish chorioretinal lesions associated with M. chimaera, from lesions due to M. tuberculosis and other infectious disorders. To date, multimodal non-invasive imaging modalities that include ultra-wide field fundus photography, fluorescein and indocyanine green angiography, optical coherence tomography and optical coherence tomography angiography, facilitate in vivo examination of retinal and choroidal tissues, enabling early diagnosis, monitoring treatment response, and relapse detection. This approach is crucial to differentiate between active and inactive ocular disease, and guides clinicians in their decisional-tree during the patients’ follow-up. In this review, we summarized and compared the available literature on multimodal imaging data of M. chimaera infection and tuberculosis, emphasizing similarities and differences in imaging patterns between these two entities and highlighting the relevance of multimodal imaging in the management of the infections.
Transcatheter aortic valve implantation (TAVI) is an alternative to open heart surgery in the treatment of symptomatic aortic valve stenosis, which is often the treatment of choice in elderly and frail patients. It carries a risk of embolic complications in the whole cerebral vascular bed, which includes the retinal vasculature. The main objective was the evaluation of retinal emboli visible on optical coherence tomography angiography (OCTA) following TAVI. This is a prospective, single center, observational study enrolling consecutive patients over two years. Patients were assessed pre- and post-TAVI. Twenty-eight patients were included in the final analysis, 82.1% were male, median age was 79.5 (range 52–88), median BCVA was 82.5 letters (range 75–93). Eight patients (28.6%) presented new capillary dropout lesions in their post-TAVI OCTA scans. There was no statistically significant change in BCVA. Quantitative analysis of macular or peripapillary OCTA parameters did not show any statistically significant difference in pre- and post-intervention. In conclusion, capillary dropout lesions could frequently be found in patients after TAVI. Quantitative measurements of macular and peripapillary flow remained stable, possibly indicating effective ocular blood flow regulation within the range of left ventricular ejection fraction in our cohort.
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