Idiopathic multifocal choroiditis (MFC): aggressive and prolonged therapy with multiple immunosuppressive agents is needed to halt the progression of active disease. An offbeat review and a case series

Background and purpose Idiopathic multifocal choroiditis (MFC) is part of the group of choriocapillaritis entities. The clinical definition of the disease has evolved with time. The aim of this article was to undertake a review on MFC, on its present-day appraisal and nomenclature and we also report a series of patients with emphasis on the clinical presentation and the importance of vigorous immunosuppressive management. Methods A review of the literature and a retrospective case series study which was performed in the Centre for Ophthalmic Specialised care (COS), Lausanne, Switzerland. Patients diagnosed from 1994 to 2020 with idiopathic multifocal choroiditis (MFC) treated with multiple immunosuppressants were included. Exclusion criteria were insufficient follow up and cases not treated with vigorous immunosuppressive therapy. Imaging analysis included spectral domain optical coherence tomography (SD-OCT) / enhanced depth imaging OCT (EDI-OCT), OCT angiography (OCT-A). Fluorescein and Indocyanine angiography (FA, ICGA) before and after the instauration of treatment. Best corrected visual acuity (BCVA), intraocular pressure (IOP), routine ocular examination, laser flare photometry (LFP) were performed at presentation and follow-up. Immunosuppression comprised at minimum two among the following agents: prednisone, cyclosporine, azathioprine, mycophenolic acid or infliximab. Mean duration of therapy was calculated. Results 26 (52 eyes) of 2102 new patients (1.24%) were diagnosed with MFC. 25 (96%) patients were female and 1 (4%) was male. 43/52 (82%) eyes were myopic with a mean dioptre of − 5.87 ± 2.94, six (12%) eyes were hypermetropic with mean dioptres 2.0 ± 2.68 and three (6%) were emmetropic. 14/52 (27%) eyes had at least 1 anti-VEGF injection because of choroidal neovascularisation (CNVs), 1 eye had a phototherapy laser and 37/52 (71%) had no complication of CNVs during the follow-up. 5/26 (19%) fulfilled the inclusion criteria for our study. Mean age was 26.4 ± 9.3 years. Snellen best corrected visual acuity (BCVA) at presentation was 0.955+/-0.26. Mean follow up was 84+/-55 months. LFP at presentation was 6.34 ± 2.94 ph/ms. None of four patients with prolonged treatment and prolonged follow-up showed disease activity. One patient still under therapy after 4 months’ follow-up still showed an active neovascular membrane. Conclusion Treatment with multiple immunosuppressive agents was shown to stop the progression of the disease.

The impact of polymerase chain reaction (PCR) on diagnosis and management of infectious uveitis at a tertiary care facility

Background Polymerase Chain Reaction (PCR) is a well-accepted adjunct in the management of infectious uveitis. In turn, few reports in the literature have evaluated how PCR then impacts patient care. This study aims to evaluate the impact of PCR sampling on diagnosis and treatment of infectious uveitidies at a large tertiary care facility. Main body This is a retrospective, observational study of patients with aqueous and vitreous PCR samples obtained from 2014 to 2019. The study was undertaken at a single institution. At least one follow up visit following results of PCR testing was required for inclusion. If a patient had multiple PCR samples taken, only the first sample was included. The patients were divided into three categories based on pre-sampling diagnosis. A chi-square test was used to analyze the data. 108 cases were available for analysis. PCR did not change diagnosis or management in any of the cases where pre-sampling diagnosis carried a high clinical suspicion for negative PCR. Overall, the results of PCR testing had a more significant impact on diagnosis in those cases where pre-sampling diagnosis was unknown versus those where it was confirmatory in nature, thus presumed to be related to an infectious entity tested by PCR (74% vs. 29%, p = 0.00006). The rate of treatment change based on PCR was similar between those cases where there was a high clinical suspicion for positive PCR and those where pre-sampling diagnosis was unknown (32% vs. 33%, p = 0.95). Further analyzing specimens separately depending on source of sample, this pattern persisted for aqueous samples, with PCR showing a more significant impact on diagnosis in those cases where the diagnosis was unknown versus those where sampling was confirmatory (86% vs. 31%, p = 0.00004). The rate of change in treatment between the two groups was similar (35% vs. 31%, p = 0.79). Vitreous samples followed a similar pattern with a higher rate of diagnosis change for those cases where pre-sampling diagnosis was unknown and a similar rate in treatment change between the two groups, however this did not reach statistical signifigance (44% vs. 25%, p = 0.28; 27% vs. 33%, p = 0.74). Conclusion There is no well-defined algorithm as to when to employ PCR testing in uveitis. As expected, in our experience, it has the largest impact on diagnosis when the diagnosis is unknown, however even when confirmatory in nature, it continues to impact patient management.

Ocular inflammatory events following COVID-19 vaccination: a multinational case series

Background Inflammatory adverse events following COVID-19 vaccination are being reported amidst the growing concerns regarding vaccine’s immunogenicity and safety, especially in patients with pre-existing inflammatory conditions. Methods Multinational case series of patients diagnosed with an ocular inflammatory event within 14 days following COVID-19 vaccination collected from 40 centres over a 3 month period in 2021. Results Seventy patients presented with ocular inflammatory events within 14 days following COVID-19 vaccination. The mean age was 51 years (range, 19–84 years). The most common events were anterior uveitis (n = 41, 58.6%), followed by posterior uveitis (n = 9, 12.9%) and scleritis (n = 7, 10.0%). The mean time to event was 5 days and 6 days (range, 1–14 days) after the first and second dose of vaccine, respectively. Among all patients, 36 (54.1%) had a previous history of ocular inflammatory event. Most patients (n = 48, 68.6%) were managed with topical corticosteroids. Final vision was not affected in 65 (92.9%), whereas 2 (2.9%) and 3 (4.3%) had reduction in visual acuity reduced by ≤3 lines and > 3 lines, respectively. Reported complications included nummular corneal lesions (n = 1, 1.4%), cystoid macular oedema (n = 2, 2.9%) and macular scarring (n = 2, 2.9%). Conclusion Ocular inflammatory events may occur after COVID-19 vaccination. The findings are based on a temporal association that does not prove causality. Even in the possibility of a causal association, most of the events were mild and had a good visual outcome.

Practice patterns regarding regional corticosteroid treatment in noninfectious Uveitis: a survey study

Background Regional corticosteroid therapy for noninfectious uveitis is well-established but usage patterns have not been studied extensively. This study aims to assess practice patterns of retina and uveitis specialists regarding their preferences on the use of local corticosteroid therapy. Methods A 13-question survey was developed regarding the practice patterns of regional corticosteroid use in specific situations and populations. The survey was distributed to both the American Uveitis Society and Macula Society. Results Responses from 87 ophthalmologists were analyzed. The two most commonly used drugs were the dexamethasone intravitreal implant (Ozurdex®) and posterior sub-tenon’s triamcinolone (also known as posterior sub-Tenon’s Kenalog, or PSTK). Regional corticosteroids were used more frequently as first-line treatment in more than half of posterior uveitis cases when compared to anterior uveitis (39.1–46.0% vs 10.3%, respectively). Respondents were more willing to use regional corticosteroids in more than half of unilateral uveitis cases than in bilateral cases (54.7% vs 18.6%, respectively). A majority of respondents (67.1%) stated that they would avoid using regional corticosteroids in patients under 8 years old. Conclusions Our results demonstrate more frequent regional corticosteroid use in posterior segment uveitis, unilateral cases, and avoidance in younger pediatric patients. Overall, the variability in these responses highlights the need for guidelines regarding regional corticosteroid use.

A case of intravascular lymphoma diagnosed with a primary vitreoretinal lymphoma-like fundus lesion

Purpose We report a case of intravascular lymphoma with primary vitreoretinal lymphoma-like fundus findings. Case A 61-year-old man with a one-week history of temporal visual field defect in the left eye was referred by a local ophthalmologist to our department. A yellowish-white raised patchy lesion was found in the nasal fundus of the left eye. Vitreoretinal lymphoma was suspected, and vitrectomy was performed in the left eye for diagnostic purpose. However, vitreous interleukin-10 concentration was low and no significant result was obtained. He had fever of around 38 °C, and respiratory failure that started 2 weeks before ophthalmological examination, worsened. Intravascular lymphoma was diagnosed from the results of histopathological examinations of transbronchial lung biopsy, bone marrow biopsy and random skin biopsy. With the start of systemic chemotherapy, the subretinal lesions shrank gradually and systemic condition was stable. However, 5 months after the start of chemotherapy, spread to the central nervous system was observed, and chimeric antigen receptor T cell (CAR-T) therapy was started in another hospital. After the start of CAR-T therapy, the subretinal lesions shrank further. Conclusions Intravascular lymphoma may be accompanied by primary vitreoretinal lymphoma-like intraocular lesions. If intraocular lesions are accompanied by systemic symptoms such as fever of unknown origin, the possibility of intravascular lymphoma should be suspected and systemic work-up should be performed.

Delayed-onset post-operative keratitis and endophthalmitis caused by Exophiala oligosperma

A case of delayed-onset post-cataract-surgery keratitis and endophthalmitis, caused by the melanin-producing fungus Exophiala oligosperma, is presented. The patient presented with an infection at the corneal side-port wound 5 months after an uneventful phacoemulsification surgery. Despite pars plana vitrectomy and combination antifungal treatment, the patient required an evisceration of the globe. Limited clinical information is available about the treatment of eye infections caused by this organism.

Multiple evanescent white dot syndrome (MEWDS): update on practical appraisal, diagnosis and clinicopathology; a review and an alternative comprehensive perspective

Background Multiple evanescent white dot syndrome (MEWDS) is a rare inflammatory eye condition affecting the outer retina as a consequence of choriocapillaris non perfusion. The pathophysiology of MEWDS will be discussed based clinical appraisal and on multimodal imaging appraisal. Methods Narrative review and perspective opinion. Results Literature review results helped us to put forward (1) the specific symptomatology (decreased/blurred vision, photopsia, subjective scotomas), (2) the ill-asserted character of clinical findings (foveal granularity, white dots in fundoscopy), (3) and the crucial importance of multimodal imaging with the diagnostic triad of ICGA hypofluorescent areas, BL-FAF hyperautofluorescent areas and loss/damage of IS/OS-ellipsoid zone on SD-OCT that characterise the disease and can practically help the clinician to diagnose MEWDS. A comprehensive alternative perspective of the disease was formulated. Conclusions The bulk of evidence that we are presenting in this review, thanks to new performing non-invasive and invasive imaging modalities, is sufficiently compelling to consider MEWDS as a primary choriocapillaritis/inflammatory choriocapillaropathy. Multimodal imaging allows the clinician to diagnose MEWDS with a high level of certainty and ensures a precise follow-up.

Orbital myiasis on recurrent undifferentiated carcinoma in the COVID-19 era: a case report and brief review of the literature

Background Myiasis is defined as the infestation of living tissues by Diptera larvae. Ophthalmic involvement occurs in less than 5% of cases. As the most uncommon type of involvement, orbital myiasis usually affects patients with poor personal hygiene, a low socioeconomic status, a history of surgery, and cancer. Findings In January 2020, an 89-year-old man presented to the Oculoplastic Department of Farabi Eye Hospital (Iran) with a history of left-side progressive orbital mass for six months. A large infiltrative mass of the left orbit with extension to the globe, periorbita, and adnexa was remarkable at the presentation, and its appearance suggested malignancy. Our findings persuaded us to perform exenteration and histopathological evaluation which were reported as “undifferentiated carcinoma”. Regular follow-up visits were recommended. In June 2020, with a 3-month delay, the patient presented with the recurrence of the mass complicated with mobile alive larva. Examinations revealed numerous maggots crawling out of an ulcerative and foul-smelling lesion. He stated that fear of COVID-19 infection postponed his follow-up visit. The patient underwent immediate mechanical removal of larvae, followed by wide local excision of the mass. Conclusion Patients with carcinoma of the adnexal tissues seem to be more prone to myiasis infestation even though it is an uncommon disease. Since COVID-19 is an ongoing pandemic with no end in sight appropriate protocols should be implemented to prevent loss of follow-up in these high risk patients.

Clinicopathology of non-infectious choroiditis: evolution of its appraisal during the last 2–3 decades from “white dot syndromes” to precise classification

Choroidal imaging investigation techniques were very limited until 2–3 decades ago.Fluorescein angiography (FA) was not suited for the analysis of the choroidal compartment and B-scan ultrasonography did not provide enough accuracy. It was on this background that a purely phenomenological approach was attempted to classify these choroiditis diseases by regrouping them under the vague potpourri term of “white dot syndromes”. With the availability of precise investigational modalities of choroidal inflammation or choroiditis-induced lesions, such as indocyanine green angiography (ICGA), spectral domain optical coherence tomography (SD-OCT) and enhanced depth imaging optical coherence tomography (EDI-OCT) it became possible to better classify these diseases based on clinico-pathological mechanisms rather than on purely phenomenological observation.Recently OCT-angiography has implemented the armamentarium of diagnostic techniques possibly also contributing to the classification of choroidal inflammatory diseases.Based on pioneering pragmatism, the aim of this article was to give a clear classification of non-infectious choroiditis. Thanks to new imaging investigations of the choroid, it is now possible to classify and understand the diverse clinicopathological mechanisms in the group of non-infectious choroiditis entities.

Lens-induced hypopyon uveitis as the presenting manifestation of posterior lens nucleus dislocation following pars-plana vitrectomy: case report

A 57-year-old otherwise healthy male presented to our department seven days following uneventful pars-plana vitrectomy with gas tamponade for a superior bullous retinal detachment in the left eye. Ophthalmic examination revealed anterior segment inflammation with hypopyon and fibrinous exudate. Intra-ocular pressure was 28 mmHg. Posterior segment evaluation was difficult to assess due to the presence of anterior capsule opacification and gas bubble. A Toxic Anterior Segment Syndrome was suspected, and the patient was treated with topical and oral corticosteroid medication in combination with anti-glaucomatous therapy. On follow-up, anterior segment inflammation and ocular hypertension improved. On day ten post-operatively, ocular ultrasonography demonstrated lens material inferiorly with attached retina. The final diagnosis of posterior lens nucleus dislocation with lens-induced uveitis was retained. The patient underwent an uneventful second vitrectomy with aspiration of the dislocated lens nucleus and sulcus three piece-lens implantation. On last follow-up, visual acuity was 20/50 with no relapsing of ocular inflammation and the retina remained reattached.