Lívio Costa’s research while affiliated with Universidade NOVA de Lisboa and other places

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune demyelinating disorder that occurs in approximately 20% of cases in a postinfectious or postvaccination context. To date, few cases of MOGAD have been reported following SARS-CoV-2 immunization. Here, we report the case of a 28-year-old female presenting with isolated unilateral optic neuritis, achieving complete recovery after corticosteroid treatment. To the best of our knowledge, this represents the second reported case of MOGAD following the mRNA-1273 vaccine and the first presenting as isolated and unilateral optic neuritis.

Purpose To report a rare case of Multicentric Castleman’s Disease presenting with bilateral panuveitis. Methods Case report. Results A 65-years-old caucasian man presented with progressive blurred vision in both eyes for two weeks, along with weight loss, polyarthralgias and reduced muscle strength persisting for about a year. Examination revealed bilateral panuveitis after intraocular lymphoma being initially considered. CT scans showed multiple lymph node enlargements suggestive of lymphoproliferative disorder. Excisional biopsy of an axillary lymph node confirmed the diagnosis of plasmacytic type Castleman’s disease. Treatment was started with topical dexamethasone and tropicamide for anterior chamber inflammation, followed by oral prednisolone. After definitive diagnosis, treatment with siltuximab was initiated, which led to significant improvement in panuveitis and systemic symptoms. The patient became off corticosteroids and continued on siltuximab with stable visual acuity and low-grade vitritis. Conclusion To the best of our knowledge we described the first case in which the diagnosis of Multicentric Castleman’s Disease was established through the investigation of bilateral panuveitis, which was successfully managed with corticosteroids and siltuximab.

Panophthalmitis is a severe condition involving not only the eye but also the surrounding orbital soft tissues. An endogenous source of panophthalmitis is rare and requires a thorough investigation to identify the infection's origin, which can be challenging. We present a rare case of a 65-year-old male patient with no systemic symptoms who presented to the emergency room with left-eye panophthalmitis, ultimately revealing occult colorectal cancer. Although vision could not be preserved, as is often the case in such severe presentations, the tumour was surgically removed, significantly improving the patient's life prognosis. This case highlights the importance of a multidisciplinary approach in managing complex patients.

Bilateral orbital metastases from breast cancer are a rare condition, occasionally posing as the initial presentation of an undetected primary tumor. Hormone receptor-positive (HR+)/HER2- negative (HER2-) breast cancer, particularly the lobular subtype, is a common primary source. Despite advancements in breast cancer treatment, managing orbital metastases remains challenging due to limited data and their association with advanced multi-system end-stage disease. This study reports the first clinical case of bilateral orbital metastases as the initial presentation of bilateral breast cancer, successfully treated with a combination of a CDK4/6 inhibitor and an aromatase inhibitor. Additionally, it comprehensively reviews and analyzes clinical features, histological subtypes, treatment strategies, and outcomes associated with bilateral orbital metastases from breast cancer. The case involves a 53-year-old woman with elevated intraocular pressure and right optic disc edema in optical coherence tomography (OCT), leading to the discovery of synchronous bilateral orbital metastases and multicentric breast nodules. These were diagnosed as metastatic HR+/HER2- breast lobular cancer, and the patient showed a remarkable response to abemaciclib and letrozole. A review of 48 patients revealed a predominantly female population (96%) with a mean age of 59 years at diagnosis. A substantial proportion (37%) had no prior cancer history at presentation, and 65% had concomitant metastases in other anatomical sites, with 15% involving intracranial metastases. Common clinical findings included limited ocular motility (58%) and vision loss (33%). Imaging often showed infiltrating orbital soft tissues (71%), with 44% involving extraocular muscles. Histologically, most cases exhibited lobular characteristics (71%). Treatment modalities ranged from radiotherapy, chemotherapy, hormone therapy, and surgery, to immunotherapy. The overall mean survival in this cohort was 12 months (ranging from 0.5 to 41 months). In conclusion, bilateral orbital metastases from breast cancer are a clinically challenging condition. This study highlights their clinical features and treatment outcomes, underscoring the need for innovative therapeutic approaches to enhance their generally poor prognosis. The presented case, where bilateral orbital metastases from bilateral breast cancer responded well to a CDK4/6 inhibitor and an aromatase inhibitor, introduces a promising treatment avenue. Further research is warranted to optimize management and improve overall outcomes for these patients.

Immune recovery uveitis (IRU) is an intraocular inflammation that typically occurs as part of immune reconstitution inflammatory syndrome (IRIS) in the eye. Typically, it affects human immunodeficiency virus (HIV)-infected patients with recognized or unrecognized cytomegalovirus (CMV) retinitis who are receiving highly active antiretroviral therapy (HAART). IRU is a common cause of new vision loss in these patients, and it manifests with a wide range of symptoms and an increased risk of inflammatory complications, such as macular edema. Recently, similar IRU-like responses have been observed in non-HIV individuals with immune reconstitution following immunosuppression of diverse etiologies, posing challenges in diagnosis and treatment. This review provides an updated overview of the current literature on the epidemiology, pathophysiology, biomarkers, clinical manifestations, diagnosis, differential diagnosis, and treatment strategies for IRU.

Background Our study aimed to assess and compare the accuracy of 8 intraocular lens (IOL) power calculation formulas (Barrett Universal II, EVO 2.0, Haigis, Hoffer Q, Holladay 1, Kane and PEARL-DGS) in patients submitted to combined phacovitrectomy for vitreomacular (VM) interface disorders. Methods Retrospective chart review study including axial-length matched patients submitted to phacoemulsification alone (Group 1) and combined phacovitrectomy (Group 2). Using optimized constants in both groups, refraction prediction error of each formula was calculated for each eye. The optimised constants from Group 1 were also applied to patients of Group 2 – Group 3. Outcome measures included the mean prediction error (ME) and its standard deviation (SD), mean (MAE) and median (MedAE) absolute errors, in diopters (D), and the percentage of eyes within ± 0.25D, ± 0.50D and ± 1.00D. Results A total of 220 eyes were included (Group 1: 100; Group 2: 120). In Group 1, the difference in formulas absolute error was significative (p = 0.005). The Kane Formula had the lowest MAE (0.306) and MedAE (0.264). In Group 2, Kane had the overall best performance, followed by PEARL-DGS, EVO 2.0 and Barrett Universal II. The ME of all formulas in both Groups 1 and 2 were 0.000 (p = 0.934; p = 0.971, respectively). In Group 3, a statistically significant myopic shift was observed for each formula (p < 0.001). Conclusion Surgeons must be careful regarding IOL power selection in phacovitrectomy considering the systematic myopic shift evidenced—constant optimization may help eliminating such error. Moreover, newly introduced formulas and calculation methods may help us achieving increasingly better refractive outcomes both in cataract surgery alone and phacovitrectomy.

Purpose: To evaluate the structural changes in the neurosensory retina (NSR) in patients with type 2 diabetes mellitus (T2DM) without diabetic retinopathy (WDR), as measured by spectral-domain optical coherence tomography (SD-OCT). Methods: Cross-sectional, non-interventional, comparative study of patients with diagnosis of T2DM WDR against matched healthy controls. Patients underwent comprehensive ophthalmic evaluation and SD-OCT examination (Spectralis SD-OCT (software version 6.0; Heidelberg Engineering). Automated retinal segmentation was performed, and measurements of overall retinal thickness (RT) as well as layer-by-layer measurements were obtained in all nine Early Treatment Diabetic Retinopathy Study (ETDRS). Enhanced-depth imaging OCT (EDI-OCT) was also performed, and choroidal thickness was measured at five locations. Generalized additive regression models were used to analyze the data. Results: A total of 175 subjects were recruited, 50 controls and 125 patients with T2DM WDR. Patients with T2DM were stratified into three groups according to disease duration: group I (<5 years, n = 55), group II (5–10 years, n = 39), and group III (>10 years, n = 31). After multivariable regression analysis, no difference in overall RT was found between patient and control groups in all ETDRS areas. However, retinal nerve fiber layer (RNFL), inner nuclear layer (INL), and retinal pigment epithelium (RPE) thickening was observed in a small number of locations. The most consistent finding throughout the ETDRS areas was thinning of the photoreceptor layer (PR). Groups I and III showed a statistically significant reduction in PR layer thickness compared to the control group (P < 0.0007). Patients in patient group I showed thinner PR layer compared to those in patient group II, and patient group III showed the thinnest PR values. The remaining retinal layers showed no statistically significant differences between patients and controls. Discussion: The findings in this study suggest that the retinal thickness profile is not linear in patients with T2DM. Although overall retinal thickness was not significantly changed compared to controls, retinal neurodegenerative changes take place at an early stage in disease, even before the microvascular changes that characterize DR are clinically apparent. In addition, photoreceptor cells are affected early in the disease process, and suggest a potential role in the early pathophysiology of DR.

Purpose: To analyze the area of retinal nonperfusion in branch retinal vein occlusion (BRVO) and its relation to retinal ganglion cell (RGC) layer thickness. Procedures: Patients with ischemic BRVO, with no signs of fluid, were evaluated by Cirrus HD-OCT-A, Zeiss. Nonperfusion findings and RGC layer thickness were evaluated in a 6x6 mm area centered in fovea. Results: 10 eyes (8 patients) were included (mean age of 75.60±12.57years min 60, max 88). Area of ischemia in deep plexus was superior to superficial plexus (p=0.013). There was a strong correlation between ischemic area of superficial and deep plexus (r=0.915; p<0.001) and an important correlation between ischemic area of superficial plexus and area of reduced RGC layer thickness (r=0.661; p=0.038). Conclusions: In ischemic BRVO, deep capillary plexus reveals a greater area of ischemia than superficial plexus. This study suggests that ischemia may cause the decrease of RGC layer thickness. Keywords: BRVO; Capillary plexus; Ischemia; OCT-A; RGC layer thickness

Purpose: To compare the thickness of all retinal layers between a nondiabetic group and diabetic patients without diabetic retinopathy (DR). Methods: Cross-sectional study, in which all subjects underwent an ophthalmic examination including optical coherence tomography. After automatic retinal segmentation, each retinal layer thickness (eight separate layers and overall thickness) was calculated in all nine Early Treatment Diabetic Retinopathy Study (ETDRS) areas. The choroidal thickness (CT) also was measured at five locations. Generalized additive regression models were used to analyze the data. Results: A total of 175 patients were recruited, 50 nondiabetic subjects and 125 diabetic patients without DR, stratified into three groups according to diabetes duration: group I (<5 years, n = 55), group II (5-10 years, n = 39), and group III (>10 years, n = 31). Overall, groups I and III of diabetic patients had a decrease in the photoreceptor layer (PR) thickness, when compared with the nondiabetic subjects in six ETDRS areas (P < 0.0007). Patients with more recent diagnosis (group I) had thinner PR than those with moderate duration (group II). Interestingly, patients with longer known disease (group III) had the thinnest PR values. There were no overall differences in the remaining retinal parameters. Conclusions: Retinal thickness profile is not linear throughout disease duration. Even in the absence of funduscopic disease, PR layer in diabetic patients seems to differ from nondiabetic subjects, thus suggesting that some form of neurodegeneration may take place before clinical signs of vascular problems arise.

Purpose: To present a retrospective study of the postoperative astigmatism in patients with DSAEK with a determination of anterior and posterior corneal astigmatism. Methods: A retrospective study of corneal astigmatism of 80 eyes of 73 patients with Fuchs Dystrophy submitted to DSAEK was performed. Postoperative total astigmatism, posterior and anterior corneal astigmatism were determined using anterior segment tomography (Scheimpflug Camera, Pentacam®), and a combined refractometer and Keratometer (Kowa KW 2000®). Central corneal thickness was evaluated with anterior segment OCT (Visante®). Careful ophthalmological follow-up examination was performed and visual acuities and refraction were recorded. Results: Patients were in average 68.35±11.58 years old and the majority were women (62.5%). Mean central corneal thickness was 545.43±36.2μm. Total corneal astigmatism was in average 1.19±0.67 diopters when evaluated by keratometry and 1.49±0.70 diopters when evaluated by corneal tomography. Posterior corneal astigmatism was in average 1.75±0.69 diopters. Anterior corneal astigmatism evaluation in these patients was associated with a considerable underestimation of total corneal astigmatism (0.31±0.12 dioptres; p<0.01). Conclusions: In patients with DSAEK, posterior corneal astigmatism is an important contributing factor for the determination of the total astigmatism. If posterior astigmatism is not considered when evaluating patients, selection of intraocular toric lenses and other refractive procedures can be incorrectly performed.