"Cornflake nevoid" lesions, mimicking choroidal lesions of neurofibromatosis 1, in the absence of neurofibromatosis 1: An imaging-based hypothesis.

Whitish hypermelanocytic flake-like lesions on scanning laser ophthalmoscopy (SLO) multicolor posterior pole imaging (PPI) can correspond to several conditions, including simple nevi or shallow choroidal melanomas, paraneoplastic fundus lesions like bilateral diffuse uveal melanocytic proliferation, or choroidal melanocytic lesions found in neurofibromatosis type 1 (NF1). We report three cases with unilateral flake-shaped choroidal lesions on SLO multicolor PPI, similar to choroidal NF1 lesions, monitored their evolution and analyzed their potential nature using multimodal imaging including SLO multicolor and classical PPI, infrared autofluorescence (IRAF), spectral-domain-optical coherence tomography (SD-OCT), enhanced-depth imaging-OCT (EDI-OCT), OCT-angiography as well as fluorescein angiography, and indocyanine green angiography (ICGA). Two oncologic patients and one healthy patient presented unilateral whitish cornflake-shaped lesions on SLO multicolor and IRAF PPI, faintly or not visible on fundus photography, hypofluorescent on the intermediate-phase ICGA, but isofluorescent on the late-phase ICGA corresponding to hyperreflective areas in the choroid immediately under the retinal pigment epithelium on SD-OCT. The lesions were nonevolutive. Multimodal imaging determined that these "nevoid" lesions were melanocytic but could not be assimilated to classical nevi, having a looser structure that allowed some indocyanine green impregnation explaining the isofluorescence on the late-phase ICGA. The lesions were similar to those described in NF1 cases and were unrelated to the oncologic status.

Prognostic value of pretreatment indocyanine green angiography in the acute uveitic phase of Vogt-Koyanagi-Harada disease.

PURPOSE: To investigate the prognostic value of pretreatment indocyanine green angiographic (ICGA) features in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease. METHODS: Retrospective analysis of 84 patients (168 eyes). Main outcome measures were final visual acuity, development of 'sunset glow fundus' (SGF) and progression to chronic recurrent evolution. RESULTS: Thirty-eight patients (76 eyes) presented in the phase preceding anterior segment (AS) inflammation (early presentation) and 46 patients (92 eyes) had AS inflammation at presentation (late presentation). The mean number of hypofluorescent dark dots (HDDs) and frequency of disc hyperfluorescence were more in the late presentation group (p < 0.001 for both comparisons), whereas the early presentation group showed higher frequencies of peripapillary punctate choroidal hyperfluorescence (p < 0.001) and hypofluorescent patches involving macula corresponding to the areas of exudative retinal detachment (p = 0.012). The mean number of HDDs and the frequency of disc hyperfluorescence were higher among eyes that developed SGF (p < 0.001 for both comparisons) and eyes that progressed to chronic recurrent evolution (p < 0.001; p = 0.001, respectively). The frequencies of peripapillary punctate choroidal hyperfluorescence and hypofluorescent patches corresponding to the areas of exudative retinal detachment were less in the eyes that developed SGF (p = 0.019; p = 0.003, respectively). Punctate choroidal hyperfluorescence elsewhere was less frequent in the eyes that developed SGF (p < 0.001) and eyes that progressed to chronic recurrent evolution (p = 0.002). CONCLUSIONS: Pretreatment ICGA has a prognostic value in initial-onset acute uveitis associated with VKH disease.

Blue-Light Fundus Autofluorescence (BAF), an Essential Modality for the Evaluation of Inflammatory Diseases of the Photoreceptors: An Imaging Narrative.

Our purpose is to describe blue-light fundus autofluorescence (BAF) features of inflammatory diseases of the outer retina characterised by photoreceptor damage. BAF from patients diagnosed with secondary and primary inflammatory photoreceptor damage were retrospectively analyzed and compared to other imaging modalities including fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT). Multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and acute syphilitic posterior placoid chorioretinitis (ASPPC), all cases corresponding to secondary photoreceptor diseases caused by inflammatory choriocapillaris nonperfusion, were included and compared to primary photoreceptor disease entities, including acute zonal occult outer retinopathy (AZOOR) and cancer-associated retinopathy (CAR). Both groups showed increased BAFs of variable intensity. In severe cases of APMPPE and ASPPC, BAF also showed hypoautofluorescent areas. In group 1 (secondary diseases) BAF hyperautofluorescent areas were associated with colocalized ICGA hypofluorescent areas, indicating choriocapillaris nonperfusion; whereas in group 2 (primary diseases), no ICGA signs were detected. The associated colocalized areas of hypofluorescence on ICGA in the first group, which were absent in the second group, were crucial to allow the differentiation between primary (photoreceptoritis) and secondary (choriocapillaritis) photoreceptor diseases. BAF patterns in inflammatory diseases of the outer retina can give relevant information on the photoreceptor and RPE involvement, with ICGA being crucial to detect concurring choriocapillaris damage and differentiating the two pathologies.

Optical Coherence Tomography Angiography (OCT-A) in Uveitis: A Literature Review and a Reassessment of Its Real Role.

BACKGROUND: The global and precise follow-up of uveitis has become possible with the availability of dual fluorescein (FA) and indocyanine green angiography (ICGA) since the mid-1990s. Progressively, additional non-invasive imaging methods have emerged, bringing value-added precision to the imaging appraisal of uveitis, including, among others, optical coherence tomography (OCT), enhanced-depth imaging OCT (EDI-OCT) and blue light fundus autofluorescence (BAF). More recently, another complementary imaging method, OCT-angiography (OCT-A), further allowed retinal and choroidal circulation to be imaged without the need for dye injection. PURPOSE: The purpose of this review was aimed at examining the evidence in published reports indicating whether OCT-A could possibly replace dye angiographic methods, as well as the real practical impact of OCT-A. METHODS: A literature search in the PubMed database was performed using the terms OCT-angiography and uveitis, OCTA and uveitis and OCT-A and uveitis. Case reports were excluded. Articles were classified into technical reports, research reports and reviews. Articles in the two latter categories were analyzed in a more detailed, individual fashion. Special attention was paid to whether there were arguments in favor of an exclusive rather than complementary use of OCT-A. Furthermore, a synthesis of the main practical applications of OCT-A in the management of uveitis was attempted. RESULTS: Between 2016 (the year of the first articles) and 2022, 144 articles containing the search terms were identified. After excluding case report articles, 114 articles were retained: 4 in 2016, 17 in 2017, 14 in 2018, 21 in 2019, 14 in 2020, 18 in 2021 and 26 in 2022. Seven articles contained technical information or consensus-based terminology. Ninety-two articles could be considered as clinical research articles. Of those, only two hinted in their conclusions that OCT-A could hypothetically replace dye methods. The terms mostly used to qualify the contribution of the articles in this group were "complementary to dye methods", "adjunct", "supplementing" and other similar terms. Fifteen articles were reviews, none of which hinted that OCT-A could replace dye methods. The situations where OCT-A represented a significant practical contribution to the practical appraisal of uveitis were identified. CONCLUSION: To date, no evidence was found in the literature that OCT-A can replace the classic dye methods; however, it can complement them. Promoting the possibility that non-invasive OCT-A can substitute the invasive dye methods is deleterious, giving the elusive impression that dye methods are no longer inevitable for evaluating uveitis patients. Nevertheless, OCT-A is a precious tool in uveitis research.

Clinical relevance of fibrin membranous structures in the intra-photoreceptor outer segment separation on spectral domain optical coherence tomography (SD-OCT) in initial-onset acute Vogt-Koyanagi-Harada disease.

PURPOSE: The purpose of the study was to investigate clinical relevance of fibrin membranous structure (FMS) in the photoreceptor outer segments on spectral-domain optical coherence tomography (SD-OCT) in untreated initial-onset acute Vogt-Koyanagi-Harada (VKH) disease. METHODS: Clinical charts of 39 patients (78 eyes) diagnosed with initial-onset VKH disease were retrospectively reviewed. Age, gender, period from the onset of symptoms to first visit, visual acuity (VA), intraocular pressure (IOP), anterior chamber cells, serous retinal detachment (SRD), SD-OCT findings, as well as fluorescein (FA), and indocyanine green angiography (ICGA) were collected. RESULTS: FMS was observed in 24 out of 39 VKH patients in either eye (61.5%). VKH patients with FMS (FMS group) were significantly younger and had the shorter period from the onset of symptoms to the first visit compared with those without FMS (non-FMS group). Mean logMAR VA and proportion of pooling of dye, mean central retinal thickness (CRT) were significantly higher in FMS group than in non-FMS group. In contrast, hyperfluorescence of the optic disc on FA was more in non-FMS group than in FMS group. Significant positive correlations between CRT and logMAR VA or IOP were only observed in the FMS group. Total amount of corticosteroids was significantly greater in FMS group than in non-FMS group. However, there were no significant differences in LogMAR VA and IOP between two groups at 6 months after treatment initiation. CONCLUSION: FMS on SD-OCT is a critical feature observed in the early stage of initial-onset acute VKH disease, which is more common in younger patients and is related to the disease activity.

Therapeutic window of opportunity in the acute uveitic phase of Vogt-Koyanagi-Harada disease: Prevention of late autoimmune complications by early intervention.

PURPOSE: To determine relationship between timing of treatment initiation and disease outcomes and whether a therapeutic window of opportunity exists in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease. METHODS: Retrospective analysis of 112 patients (224 eyes). Main outcome measures were final visual acuity, progression to chronic recurrent evolution, development of complications, particularly 'sunset glow fundus', and drug-free remission cure of uveitis. RESULTS: Forty-six patients (92 eyes) presented in the phase preceding anterior segment (AS) inflammation (early presentation) and 66 patients (132 eyes) had AS inflammation at presentation (late presentation). In significantly more eyes in the early presentation group (85.9%), final visual acuity of 20/20 was achieved compared with those in the late presentation group (66.7%) (p = 0.001). None of the eyes in the early presentation group progressed to chronic recurrent evolution and none developed 'sunset glow fundus', whereas in the late presentation group, 28.8% of the eyes progressed to chronic recurrent evolution (p < 0.001) and 56.1% developed 'sunset glow fundus' (p < 0.001). Patients in the early presentation group were able to discontinue treatment without relapse of inflammation at significantly shorter time intervals compared to patients in the delayed presentation group (p < 0.001). In the late presentation group, logistic regression analysis demonstrated that presenting clinical features predicting unfavourable outcomes were posterior synechiae (odds ratio = 4.03; 95% confidence interval [CI] = 1.29-12.23), bullous exudative retinal detachment extending to the periphery (odds ratio = 3.35; 95% CI = 1.53-7.32) and female gender (odds ratio = 2.05; CI = 1.08-3.90). CONCLUSIONS: Our findings suggest that the window of opportunity lies in the phase preceding AS inflammation and initiation of effective treatment during this phase results in cure of uveitis and prevents blinding complications.

Benefits and Limitations of OCT-A in the Diagnosis and Follow-Up of Posterior Intraocular Inflammation in Current Clinical Practice: A Valuable Tool or a Deceiver?

PURPOSE: Optical coherence tomography angiography (OCT-A) has been applied to uveitis and intraocular inflammation since its availability after 2014. The imaging of retinal and choroidal vascularization without the use of dyes was a major development and represented a potentially valuable tool in ocular research. In addition to such use, OCT-A is often put forward as being able to potentially replace invasive methods needing dye injection, such as fluorescein angiography (FA) and indocyanine green angiography (ICGA). The aim of this review was to establish whether OCT-A was sufficiently useful in everyday routine clinical practice to monitor disease evolution and to perform treatment adjustments to the extent that it could reliably replace the standard dye methods. METHODS: Selective literature review and analysis of own data and experience. RESULTS: OCT-A is a technologically high-grade imaging modality allowing to analyze retinal circulation in inflammatory diseases of the posterior pole with a high sensitivity useful for research purposes. However, there is no evidence that it reaches equal effectiveness in the routine management of posterior uveitis involving the retina. OCT-A is unable to show leakage. In choriocapillaritis involving pre-capillary vessels, it shows capillary drop-out but does not seem to have an advantage over ICGA except that it can be repeated easily, not being invasive, and so allows a closer follow-up. It is, however, less useful in end-choriocapillary non-perfusion, such as in MEWDS. For choroidal stromal inflammation, OCT-A is ill-suited as it only shows inconsistent secondary circulatory changes produced by choroidal foci. OCT-A seems to be useful in the diagnosis and follow-up of inflammatory chorioneovascularisation (iCNV), although dye exams are more precise in showing the activity of the iCNV. CONCLUSION: In summary, OCT-A is a very sensitive modality for the retinal circulation in uveitis for research purposes; it is sometimes useful for close follow of choriocapillary drop-out but not in end-capillary non-perfusion. Its use for monitoring purposes in stromal choroiditis, however, is questionable. Its claim to possibly replace classical angiographic work-up for the practical management of posterior uveitis is largely overrated.

Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging Perspective.

BACKGROUND AND AIM: Inflammatory diseases that affect the outer retina do so by different mechanisms. Some of them result from the direct, primary involvement of the outer retina (primary photoreceptoritis) such as acute zonal outer occult retinopathy (AZOOR). Others affect the photoreceptors secondarily due to the inflammatory involvement of the choriocapillaris. This results in choriocapillaris non-perfusion that damages the photoreceptors due to the ensuing ischaemia, a mechanism characterising primary inflammatory choriocapillaropathies (PICCPs) such as multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), and others. Thanks to multimodal imaging (MMI), it is now possible to differentiate between these two mechanisms of outer retinal damage. The aim of this study is to determine the MMI characteristics that allow us to differentiate primary photoreceptoritis, including AZOOR, from PICCPs such as MEWDS and MFC. METHODS: A series of eight PICCPs cases (five typical MEWDS and three typical active MFC cases) and four typical primary photoreceptoritis/AZOOR cases (five eyes) that had undergone complete MMI investigation, including fundus photography (FP), blue light fundus autofluorescence (BL-FAF), spectral domain optical coherence tomography (SD-OCT), OCT angiography (OCT-A, when available), fluorescein angiography (FA), and indocyanine green angiography (ICGA) were analysed, pointing out the differences that allow us to distinguish primary photoreceptoritis from PICCPs. RESULTS: All primary photoreceptoritis/AZOOR cases showed (1) faint fundus pallor around the fovea, (2) BL-FAF hyperautofluorescence, (3) loss of photoreceptor outer segments (PROS) on SD-OCT, (4) absence of choriocapillary drop-out on OCT-A, (5) normal FA or faint FA hyperfluorescence, and (6) conserved ICGA fluorescence/no hypofluorescent areas; (1), (2), (3), and (5) indicated loss of photoreceptor outer segments, and (4) and (6) indicated conserved choriocapillaris circulation. For PICCPs, (a) fundus showed discreet white dots or none (in MEWDS) and punched-out scars in MFC, (b) BL-FAF hyperautofluorescence, (c) loss of PROS on SD-OCT, (d) FA faint hyperfluorescence in MEWDS, also minimal in active MFC lesions (e) in all cases ICGA hypofluorescent areas; (b) and (c) indicating loss of PROS, and (e) indicating choriocapillaris non-perfusion in all cases. The OCT-A did not show consistent findings with faint or no capillary drop-out in MEWDS and MFC. CONCLUSIONS: MMI combining the SD-OCT and BL-FAF clearly showed loss of PROS in both groups, while the ICGA determined whether this was due to choriocapillaris non-perfusion in PICCPs or whether the choriocapillaris was intact in case of primary photoreceptoritis. The FA and OCT-A were found to be less useful and/or less sensitive for the appraisal of both these entities.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): A Comprehensive Approach and Case Series: Systemic Corticosteroid Therapy Is Necessary in a Large Proportion of Cases.

Background and objectives: Acute posterior multifocal pigment epitheliopathy/acute multifocal ischaemic choriocapillaritis (APMPPE/AMIC) is part of the group of choriocapillaritis entities. The aim of this article was to report a series of patients with emphasis on the clinical presentation and treatment paradigms. Materials and Methods: Retrospective case series study performed in the Centre for Ophthalmic Specialised care (COS), Lausanne, Switzerland, on patients diagnosed from 2000 to 2021 with APMPPE/AMIC. Procedures performed at presentation and upon follow-up (when available) included best corrected visual acuity (BCVA), routine ocular examination, laser flare photometry (LFP) microperimetry (when available) and visual field testing. Imaging investigations included spectral domain optical coherence tomography (SD-OCT)/enhanced depth imaging OCT (EDI-OCT), OCT angiography (OCT-A) as well as fluorescein and indocyanine green angiography (FA, ICGA). The presence or not of prodromal systemic viral-like symptoms was noted. The localisation of lesions whether foveal or extrafoveal, divided the patients into 2 groups (foveal, peri-or parafoveal). Exclusion criteria were patients diagnosed with APMPPE/AMIC and a positive QuantiFERON test and/or VDRL-TPHA tests. Results: Nineteen (35 eyes) of 1664 new patients (1.14%) were diagnosed with APMPPE/AMIC and included in our study. 13 (68%) were male and 6 (32%) were female. The mean age was 33.1 ± 9.2 years. 16 (84%) patients mentioned a viral prodromal episode or other systemic symptoms, and 3 (16%) did not mention any episode before the onset of ocular symptoms. 15 (39%) out of 38 eyes had foveal localisation of the lesions, 20 (52.6%) had peri- or para-foveal localisations and 3 eyes were normal [3 unilateral cases (15%)]. Mean BCVA at presentation was 0.83 ± 0.24 for the whole group. It was 0.58 ± 0.28 for the group with foveal lesions, increasing to 0.97 ± 0.13 at last follow-up (p = 0.0028). For the group with extrafoveal lesions mean BCVA at presentation was 0.94 ± 0.18, improving to 1.18± 0.10 at last follow-up (p = 0.0039). 13 (68%) patients received prednisone treatment, of whom 2 (10%) received additionally at least one immunosuppressive agent, 4 (20%) patients received no treatment and in 2 patients the information was unavailable. All patients in the foveal lesion group received corticosteroid treatment except one who evolved to bilateral macular atrophy. Conclusions: APMPPE/AMIC is a primary choriocapillaritis. Although it is thought that the disease is self-limited, treatment is necessary in most cases, especially when lesions are located in the fovea.

Efficacy and Safety of Adalimumab for Exacerbation or Relapse of Ocular Inflammation in Patients with Vogt-Koyanagi-Harada Disease: A Multicenter Study.

PURPOSE: We investigated efficacy and safety of adalimumab (ADA) treatment for exacerbation or recurrence of Vogt-Koyanagi-Harada (VKH) patients. METHODS: Medical records of 70 VKH patients who received ADA treatment for more than 6 months were retrospectively investigated. RESULTS: The mean age of VKH patients was 54.8 ± 15.1 years, and male/female ratio was 34/36, and sunset glow fundus was observed in 71.4%. Subfoveal choroidal thickness, indocyanine green angiography scores, and corticosteroid and cyclosporine doses were significantly reduced by ADA treatment for 6 months compared to baseline, while LogMAR and flare counts were also improved without being statistically significant. Adverse events were observed in 17.1%, in which tuberculosis was at 7.14% and psoriasis was at 2.86%; however, ADA treatment was continued in 91.4%. CONCLUSIONS: ADA was shown to be effective to achieve remission of VKH disease refractory to conventional treatments and was generally well tolerated with few serious adverse events.

Vogt-Koyanagi-Harada disease: the step-by-step approach to a better understanding of clinicopathology, immunopathology, diagnosis, and management: a brief review.

BACKGROUND: Appraisals of Vogt-Koyanagi-Harada disease (VKH) have become progressively more complete, since its first description in 1906. The availability of new investigational methods has improved our knowledge of the immunopathology, clinicopathology, diagnosis, and management of VKH disease. This review aimed to describe some of the steps that led to better characterization of VKH as a clinical entity. METHODS: We searched on PubMed for articles that described the history of VKH disease and analyzed the progress in disease appraisal with new investigational and imaging methods. In particular, we searched for articles that investigated the clinicopathology, diagnosis, and management of VKH. FINDINGS: The following developments were considered essential for improving the appraisal and understanding of VKH: (1) the history of the disease, (2) immunopathological mechanisms, (3) clinicopathology, (4) the importance of distinguishing initial-onset from chronic disease, (5) relevant imaging modalities, among which indocyanine green angiography is crucial, (6) diagnostic criteria that facilitate early diagnosis, and (7) the need for early, prolonged, aggressive treatment that combines steroidal and non-steroidal immunosuppression. CONCLUSION: Based on these findings, the definition of VKH has improved. VKH disease starts in the choroidal stroma and later involves other structures when it is not diagnosed and treated early. Indocyanine green angiography and enhanced depth imaging optical coherence tomography facilitate early diagnosis and precise monitoring of choroidal inflammation. ICGA is clearly the gold standard for appraisals and follow-ups in VKH disease, however EDI-OCT should be especially considered in those areas where ICGA is not fully available. These modalities have contributed substantially to a "cure" for VKH, when treatment is introduced within the therapeutic window of opportunity.

Mechanisms, Pathophysiology and Current Immunomodulatory/Immunosuppressive Therapy of Non-Infectious and/or Immune-Mediated Choroiditis.

Non-infectious choroiditis comprises immune-mediated diseases resulting from diverse pathophysiological mechanisms. These conditions are sub-divided into two main groups, (1) diseases of the choriocapillaris and (2) diseases of the choroidal stroma. The purpose of this study is to expose the pathophysiology of the most common diseases of both these groups and recommend the optimal immunomodulatory/immunosuppressive therapy of each analyzed condition based on literature data and data from our own centers. Material and Methods: Narrative review. In the group of choriocapillaritis entities or primary inflammatory choriocapillaropathies (PICCPs) including multiple evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), idiopathic multifocal choroiditis (MFC) and serpiginous choroiditis (SC), as well as secondary choriocapillaritides including acute syphilitic posterior multifocal placoid chorioretinitis (ASPMPC) and tuberculosis-related SC (TB-SC), were analyzed. In the group of stromal choroidites, HLA-A29 birdshot retinochoroiditis (BRC) and Vogt-Koyanagi-Harada (VKH) disease were included. For each entity a literature search, in the PubMed database, on treatment was performed and analyzed and the therapeutic attitudes of our own centers were presented. Management of immune-mediated choroiditis implies vigorous immunosuppressive therapy given in a prompt and prolonged fashion in most of these entities.

Pituitary Macroadenoma: Fooling the Ophthalmologist before Giving Him the Role of the Whistle-Blower.

PURPOSE: To describe cases diagnosed with pituitary macroadenoma during the follow-up of their primary ocular pathologies. METHODS: Charts of patients followed in the Centre for Ophthalmic Specialized Care for diverse pathologies and who had subsequently developed a pituitary macroadenoma were retrieved. The primary pathologies were noted. The delay of the diagnosis after sufficiently compatible perimetric signs became available was calculated and the evolution after the neurosurgical intervention was reported. RESULTS: In total, from 2003 to 2020, 16/14 966 (0.1%) pituitary macroadenoma patients were recorded. In 10 patients, the disease was noted in their history. In 6 patients (2 females, 4 males; 0.04%), macroadenoma occurred during the follow-up for their primary ocular pathologies. Mean age at first presentation was 65.16 ± 8.52 years. Primary pathologies included amblyopia (1), glaucoma (2), cataract (4), and uveitis (2). Mean duration of symptoms was 18.17 ± 13.11 months. Mean delay from first suspicious visual field signs to diagnosis was 125 ± 207.93 days. All patients underwent one surgical treatment with or without radiotherapy except one where a second intervention was required. All patients have seen their visual field improve after surgical intervention. Mean preoperative Mean defect (MD) was 13.43 ± 8.68 dB OD and 13.4 ± 5.07 dB OS. Mean postoperative MD was 8.2 ± 10.27 dB OD and 5.42 ± 4.12 dB OS. CONCLUSION: Pituitary macroadenomas are prone to be missed or diagnosed with delay when ophthalmic patients are already followed for another pathology that prevents the clinician from diligently evoking the diagnosis. Despite profound visual field loss, visual recovery was almost complete in 4/6 patients, indicating that even diagnostic delay did not preclude recovery in our series.

Diagnosis and Treatment of Primary Inflammatory Choriocapillaropathies (PICCPs): A Comprehensive Overview.

PURPOSE: Primary inflammatory choriocapillaropathies (PICCPs) belong to a group of intraocular inflammatory diseases with the common characteristic of inflammatory choriocapillaris hypo- or non-perfusion as the main clinicopathological mechanism. The purpose of our article is to describe clinical characteristics and multimodal imaging, that can help the diagnosis and treatment of PICCPs. METHODS: Narrative review with multimodal imaging analysis. RESULTS: Choriocapillaris non-perfusion can affect the end-choriocappilaries, at the benign end of the PICCP spectrum (MEWDS), to larger choriocapillaris vessels or precapillary vessels at the origin of more severe forms such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE), idiopathic multifocal choroiditis (MFC) and Serpiginous Choroiditis (SC). Diagnosis is mostly based on multimodal imaging and especially on indocyanine green angiography (ICGA), fundus autofluorescence (FAF) and spectral-domain optical coherence tomography (SD-OCT)/OCT-angiography (OCT-A). ICGA shows the typical pattern of patchy lobular hypofluorescence reflecting hypo- or non-perfusion of the choriocapillaris that can also take the aspect of geographic areas in the more severe forms. Treatment depends on the severity of the disease and goes from observation in MEWDS and some mild cases of APMPPE, to oral corticosteroid and/or immunomodulator agents in the more severe conditions of APMPPE and MFC and SC cases. Close multimodal monitoring is crucial in order to introduce or adjust treatment. CONCLUSION: PICCPs are resulting from one common clinicopathological mechanism, inflammatory choriocapillaris hypo- or non-perfusion. ICGA findings are essential for the diagnosis and follow-up of PICCPs, but non-invasive methods such as FAF and SD-OCT/OCT-A also have their role especially in follow-up of the diseases. Treatment should be individualized according to the pathology and the evolution of lesions.

Tuberculosis-related serpiginous choroiditis: aggressive therapy with dual concomitant combination of multiple anti-tubercular and multiple immunosuppressive agents is needed to halt the progression of the disease.

BACKGROUND/PURPOSE: Serpiginous-like choroiditis is a rare immune-mediated sub-entity of tubercular uveitis with a usually deleterious outcome. Treatment is still controversial. The purpose in this case series is to indicate that only aggressive treatment comprising multiple anti-tubercular and multiple immunosuppressive agents seems to be able to halt the disease progression. METHODS: This retrospective case series included patients diagnosed with Interferon Gamma Release Assays (IGRA) -positive serpiginous choroiditis, seen at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland, treated with combined multiple antitubercular and immunosuppressive agents at presentation and having a sufficient follow-up. Disease history before referral, appraisal of disease, treatment modalities and follow-up were analyzed. Inclusion criteria were positive IGRA patients with serpiginous choroiditis with complete Spectral-Domain Optic coherence tomography (SD-OCT) and angiography images. RESULTS: From 2001 to 2020, 24 of 1525 new patients (0.26%) were diagnosed as serpiginous choroiditis. 10/24 were related to tuberculosis (positive IGRA and/or hyper-positive Mantoux test), 8/24 were IGRA negative and in 6 there was no information available. 4/10 tuberculosis related serpiginous patients fulfilled the inclusion criteria. Mean age was 39 ± 5.3 years. Snellen best corrected vision acuity (BCVA) at presentation in 3/4 where the macula was preserved was 0.96 ± 0.08. In 3/4 patients, treatment with multiple tuberculostatic therapy combined with multiple immunosuppressive agents, started at presentation or in the initial months after the first consultation, was shown to stop the progression of the disease, with a retained visual acuity of 1.0. One patient with macular involvement and a bilateral visual acuity of hand movements after 11 years of insufficient treatment, improved his visual acuity to 0.25 OD and 0.05 OS and presented a substantial visual field improvement that stabilized once multiple anti-tubercular and immunosuppressive therapy was introduced. CONCLUSION: IGRA-positive serpiginous choroiditis (serpiginous-like choroiditis) could be halted by combined multiple tuberculostatic and multiple immunosuppressive agents, as seen in our study where 3/4 early treated patients had conserved central function and one late treated patient had recovered a substantial amount of visual field. In all 4 patients this treatment regimen halted the progression of the disease.

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.