Fingerprint sign in Vogt-Koyanagi-Harada disease: a case series

Background The tomographic finding, which has been called the "fingerprint sign" in en face reconstructions, seems to be the result of a variety of processes that cause distension of the outer plexiform layer (OPL) and the Henle fiber layer (HFL). The aim of this paper is to describe the appearance of concentric rings at the OPL/HFL interface visualized using en face reconstructions of cross-sectional optical coherence tomography images of patients with Vogt-Koyanagi-Harada disease. Methods Retrospective analysis of images of six eyes of three patients obtained by cross-sectional OCT imaging and en face reconstruction at the level of the OPL/HFL interface. Results All eyes presented with a dentate or saw-tooth pattern of the OPL/HFL interface on cross-sectional OCT with corresponding concentric rings on en face OCT reconstruction, consistent with the recently published “fingerprint sign”. Initial OPL/HFL interface changes were observed between the first and fourth months after treatment and resolution of VKHD associated serous retinal detachments. These OPL/HFL interface changes have persisted for many years following the resolution of the active inflammation. Conclusions Changes in the OPL/HFL interface can be identified following successful treatment of VKHD. These included both a dentate or saw-tooth pattern on cross-sectional imaging and concentric rings or the “fingerprint sign” on en face reconstructions. These changes persisted for many years despite disease quiescence.

Choroidal changes and associations with visual acuity in diabetic patients

Background The variable visual function observed in diabetic retinopathy (DR) patients is not fully explained by the classic staging system. Our purpose was to evaluate choroidal changes, in standardized sectors, in DR patients and to find associations between choroidal measurements and visual function. Methods Cross-sectional study that included the right eye of diabetic patients (n = 265) without active edema, ischemia or neovascularization and age-matched controls (n = 73). Optical coherence tomography (OCT) imaging was performed with enhanced depth imaging protocol. Choroidal vascularity index (CVI) was calculated in a 5 mm scan centered in the fovea. Results CVI decreased with age (p < 0.001) but was not influenced by axial length. A multivariate analysis adjusting for age confirmed a significant difference in CVI between DR eyes that had previous treatments (intravitreal injections and/or photocoagulation) compared to control eyes (p = 0.013) and to DR eyes that never required treatment (p = 0.002). There was no significant difference between non-DR diabetic patients and normal controls. Considering the group of DR patients that had previous treatments, in eyes without optic media opacification, BCVA correlated with CVI (r = − 0.362, p < 0.001), whereas full retina thickness and individual retinal layer thickness did not (p > 0.066). Conclusions A reduction in CVI was observed in patients with a more advanced stage of DR. In treated DR patients with stable disease, choroidal biomarkers correlated with best-corrected visual acuity whereas retinal biomarkers did not. Trial registration: N/A

Birdshot retinochoroiditis in Brazil: a multicenter review of 40 patients

Background Birdshot retinochoroiditis (BRC) is a rare and chronic bilateral uveitis mostly found in Caucasians. As few data are available about the clinical course of BRC in Hispanic patients, we aimed to report the clinical findings and the evolution of BRC in Brazilian patients. Methods This retrospective cohort multicenter nationwide study was performed by analyzing the records of patients with BRC diagnoses from Brazilian ophthalmological centers from April 1995 to May 2020. Results Forty patients (80 eyes) with a diagnosis of BRC were evaluated. The mean age was 53 years, and there was no sex predominance. All tested patients (34/40) were positive for HLA-A29. The diagnosis of BRC was made following the Levinson et al. criteria, and all ancillary tests were performed to exclude differential diagnoses. Clinical signs and symptoms, such as complications and treatment, were described. Conclusions BRC evolution in Brazilian patients seems to have some peculiarities that diverge from the published literature available about Caucasians, as AS inflammation is higher in this population.

Conjunctival lymphangiectasia and retinal angiopathy in hereditary transthyretin amyloidosis

Background Hereditary transthyretin amyloidosis (ATTR amyloidosis) is a rare condition where a mutation in the transthyretin gene leads to systemic deposition of amyloid. The manifestations and prognosis of ATTR amyloidosis depends on the specific ATTR mutation, with over 100 mutations reported in the literature. The manifestations of many rare forms of ATTR amyloidosis have not been well described, particularly the late-onset ophthalmic findings. Case presentation We present the case of a 43-year-old Caucasian male with a diagnosis of ATTRD18E amyloidosis confirmed by fat pad biopsy. He had diffuse systemic involvement, including cardiovascular, pulmonary, and gastrointestinal symptoms. He also had significant ocular involvement including vitreous opacities, retinal angiopathy, and conjunctival lymphangiectasia. These ocular findings modestly progressed at 2-year follow-up. Discussion The ATTRD18E mutation is a rare variant, with few described cases. To our knowledge, this is the first documented case of ATTRD18E amyloidosis with significant ocular involvement. These ocular findings may serve as a relevant biomarker for severe disease prognosis in ATTRD18E amyloidosis. With improving treatments addressing the systemic symptoms of ATTR amyloidosis, a better understanding of the late-onset ocular symptoms is becoming increasingly relevant.

Eales’ disease: epidemiology, diagnostic and therapeutic concepts

Background To describe the epidemiological traits, clinical characteristics, diagnostic procedures, therapeutic interventions and evolution in a large series of patients with diagnosis of Eales’ disease. Methods A clinical retrospective review of patients with Eales’ disease, evaluated and treated between April 2009 and April 2018, with a 1-year minimum follow-up. Thirty patients (59 eyes), were included. Age, sex, laboratory results (CBC, glycemia, protein electrophoresis, ACE levels) immunological profile and a Quantiferon-TB Gold Plus test were recorded. The patients were divided into groups according to their evolution, medical or surgical treatment, and visual outcomes. Results Seventeen male patients and 13 female patients were included, and their ages ranged from 14 to 35 years. The Quantiferon-TB Gold Plus test was positive in 25 patients. Twenty-eight patients had unilateral vitreous hemorrhage, 10 of whom presented with vasculitis and non-perfusion areas in the contralateral eye, 9 presented contralateral peripheral neovascularization and 9 had contralateral fibrovascular proliferation. The remaining 2 patients presented with a rhegmatogenous retinal detachment. In 6 patients, conservative treatment with intravitreal anti-VEGF injections and photocoagulation was performed after the hemorrhage cleared. Twenty-two patients, required vitrectomy, with good visual outcomes. Macular edema was found in 16 eyes, which responded to periocular and/or systemic corticosteroid therapy, except for 9 eyes that required intravitreal bevacizumab, with complete resolution in 7 eyes and partial resolution in 2 eyes. Conclusions Eales’ disease is a pathology of significant prevalence in our country. The distribution according to sex, tends to be equivalent. The etiology, even when it is not specifically determined, according to laboratory tests, confirms the probable immunologic response in the presence of Mycobacterium tuberculosis antigens. This is still a diagnosis of exclusion, and therefore, it is advisable to perform a complete laboratory work-up in each case. Timely application of laser and other medical treatments, help to avoid progression to more advanced stages and their complications. The surgical treatment of vitrectomy for vitreous hemorrhage, and/or tractional vitreous detachment yields good primary anatomical and functional outcomes. Secondary macular edema responds to periocular and intravitreal corticosteroids, and in refractory cases, the use of anti-VEGF therapy leads to an effective resolution.

Future of anti-VEGF: biosimilars and biobetters

The advent of Anti- VEGFs like Lucentis (Ranibizumab), Eylea (Aflibercept) and off-label Avastin (Bevacizumab) have radically improved visual outcomes in patients of neovascular Age Related Macular Degeneration (nARMD), Diabetic Macular Edema (DME) and Retinal Vein Occlusion (RVO). It is a matter of great concern that the US patents for Ranibizumab and Aflibercept expired in 2020 with European patents to expire in 2022 and 2025, respectively. With the expiry of these biologics, Biosimilars can prove to be saviours in the posterior segment pharmacotherapy owing to their cost effectiveness and availability of various options. Numerous biosimilars are expected to gain approval for clinical use from the US-FDA and EMA soon. Biobetters are better than the original biologic in one or more parameters but require more research and development resources. With the emergence of better manufacturing and purification processes it is imperative that the biologics and biosimilars become better. The Ophthalmologists need to have in depth knowledge about these Biosimilars and Biobetters before these molecules take over the mainstream market.

Diabetic retinopathy classification for supervised machine learning algorithms

Background Artificial intelligence and automated technology were first reported more than 70 years ago and nowadays provide unprecedented diagnostic accuracy, screening capacity, risk stratification, and workflow optimization. Diabetic retinopathy is an important cause of preventable blindness worldwide, and artificial intelligence technology provides precocious diagnosis, monitoring, and guide treatment. High-quality exams are fundamental in supervised artificial intelligence algorithms, but the lack of ground truth standards in retinal exams datasets is a problem. Main body In this article, ETDRS, NHS, ICDR, SDGS diabetic retinopathy grading, and manual annotation are described and compared in publicly available datasets. The various DR labeling systems generate a fundamental problem for AI datasets. Possible solutions are standardization of DR classification and direct retinal-finding identifications. Conclusion Reliable labeling methods also need to be considered in datasets with more trustworthy labeling.

The effect of silicone oil tamponade on retinal layers and choroidal thickness in patients with rhegmatogenous retinal detachment: a systematic review and meta-analysis

Background To evaluate the effects of intravitreal silicone oil (SO) on the retinal and choroidal thickness in eyes with rhegmatogenous retinal detachment (RRD). Methods A literature search was performed in Web of Science, Scopus, ProQuest, Embase, Clinical Key, Science Direct, Cochrane Library, and Springer, as well as Persian databases, including IranDoc, MagIran, SID, MOH thesis, and MOH articles until June 2020. Two reviewers independently searched and extracted the data. Results Sixteen studies (n = 391) met the inclusion criteria. The meta-analysis showed that the SO tamponade could significantly reduce the central macular thickness (CMT) in patients with RRD as compared to gas tamponade WMD = − 14.91; 95% CI: − 22.23, − 7.60; P < 0.001, I2 = 71%). No significant change was found in CMT between the eye with SO tamponade (after SO removal) and the fellow healthy eye in patients with RRD (WMD = − 3.52; 95% CI: − 17.63, 10.59; I2 = 68.6%). Compared to the preoperative stage, the SO tamponade could significantly reduce the subfoveal choroidal thickness in patients with RRD (WMD = − 18.67, 95% CI: − 30.07, − 1.28; I2 = 80.1%). However, there was no significant difference in the subfoveal choroidal thickness before and after SO removal (WMD = − 1.13, 95% CI: − 5.97, 3.71; I2 = 87.6%). Conclusion The SO tamponade had a significant effect on the reduction of retinal layers and the subfoveal choroidal thickness.

Update on surgical management of complex macular holes: a review

Modern surgical interventions effectively treat macular holes (MHs) more than 90%. Current surgical treatment for MHs is pars plana vitrectomy with epiretinal membrane, internal limiting membrane (ILM) peeling, gas endotamponade, and prone posturing postoperatively. However, a small subset of MHs imposes challenges to surgeons and frustrations on patients. A narrative review was performed on the surgical treatment of challenging MHs including large and extra-large MHs, myopic MHs with or without retinal detachment, and chronic and refractory MHs. There are robust data supporting inverted ILM flap as the first-line treatment for large idiopathic MHs and certain secondary MHs including myopic MHs. In addition, several studies had shown that ILM flap manipulations in combination with surgical adjuncts increase surgical success, especially in difficult MHs. Even in eyes with limited ILM, surgical options included autologous retinal graft, human amniotic membrane, and creation of a distal ILM flap that can assist in MH closure even though the functional outcome may be affected by the MH chronicity. Despite relative success anatomically and visually after each technique, most techniques require a long-term study to analyze their safety profile and to establish any morphological changes of the MH plug in the closed MHs.

Early neurovascular retinal changes detected by swept-source OCT in type 2 diabetes and association with diabetic kidney disease

Purpose To evaluate retinal thickness and capillary density in patients with type 2 diabetes (T2D) and their association with diabetic kidney disease (DKD) using swept-source optical coherence tomography (SS-OCT). Methods A cross-sectional study was conducted with T2D patients with mild or no diabetic retinopathy (DR) and nondiabetic controls. Inner retinal layer thickness was measured with SS-OCT. Retinal capillary density and the foveal avascular zone (FAZ) were measured with SS-OCT angiography (OCTA). SS-OCT parameters were compared in patients with and without diabetic kidney disease (DKD) and nondiabetic controls. Results 131 DKD eyes showed decreased ganglion cell layer plus (GCL+) (p = 0.005 TI; p = 0.022 I), retinal nerve fiber layer (RNFL) (p = 0.003), and central retinal thickness (CRT) (p = 0.032), as well as foveal avascular zone (FAZ) enlargement (p = 0.003) and lower capillary density in the superficial vascular plexus (p = 0.016, central quadrant), compared to controls. No statistically significant changes were found between diabetic patients without significant DKD and controls. Conclusion Our findings suggest early neurovascular damage in patients with T2D; these changes were more significant in patients with DKD. Larger longitudinal studies are warranted to determine the role of early neurovascular damage in the pathophysiology of severe DR.