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.

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.

Initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease presenting with unilateral exudative retinal detachment despite bilateral choroidal involvement.

PURPOSE: To investigate the frequency of initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease presenting with unilateral exudative retinal detachment. METHODS: A retrospective case series. RESULTS: During the period between January 1998 and December 2020, we identified 135 patients with initial-onset acute uveitis associated with VKH disease. Among them, 5 (3.7%) patients were referred to have unilateral uveitis due to the presence of exudative retinal detachment in only one eye. Optical coherence tomography confirmed the presence of unilateral exudative retinal detachment, however, indocyanine green angiography (ICGA) revealed characteristic findings of bilateral granulomatous choroidal inflammation typical for initial-onset acute uveitis associated with VKH disease. CONCLUSIONS: Patients with initial-onset acute uveitis associated with VKH disease can present with unilateral exudative retinal detachment. ICGA assessment of the choroid revealed the presence of subclinical involvement of the fellow eyes.

Vogt-Koyanagi-Harada disease: recurrence rates after initial-onset disease differ according to treatment modality and geographic area.

Background/Purpose Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune stromal choroiditis producing a spill-over panuveitis. For initial-onset VKH disease, it is increasingly thought that corticosteroid therapy is not sufficient and additional non-steroidal immunosuppressive therapy is needed. At the 11th workshop on VKH, the disease was said to be well controlled with corticosteroids alone in Japanese patients. The aim of this study was to review the literature to determine whether different levels of severity exist in different geographical areas. METHODS: Literature was reviewed for studies on the evolution of initial-onset VKH disease, looking at treatment modalities and proportion of cases with chronic evolution and/or sunset-glow fundus (SGF). RESULTS: PubMed search yielded 1249 references containing the term of Vogt-Koyanagi-Harada. Twenty references (15 from outside of Japan and 5 from Japan) contained information on the evolution of treated initial-onset disease. For the "international" group, percentage of chronic evolution after systemic corticosteroid monotherapy was 61%, and after combined steroidal and non-steroidal therapy it fell to 2% (0% in 3/4 studies). In the Japanese studies where all patients received systemic corticosteroids alone, chronic evolution was reported in 25%; however, SGF amounted to 61%. CONCLUSION: In the world at large, chronic evolution of initial-onset VKH disease treated with corticosteroids alone concerned two-thirds of patients. Japanese studies showed that chronic evolution was substantially less frequent, indicating possibly less severe disease in Japan. This proportion fell to almost zero when dual steroidal and non-steroidal immunosuppression was given at onset.

Vogt-Koyanagi-Harada Disease and Birdshot Retinochoroidopathy, Similarities and Differences: A Glimpse into the Clinicopathology of Stromal Choroiditis, a Perspective and a Review.

The purpose of this work was to give a comprehensive and updated review on two primary stromal choroiditis entities, Vogt-Koyanagi-Harada disease (VKH) and birdshot retinochoroiditis (BRC). Their appraisal has become much more precise thanks to new investigational methods, such as indocyanine green angiography (ICGA) and enhanced depth imaging optical coherence tomography (EDI-OCT), which give substantially improved imaging access to the choroid. In this review, we focus on the crucial changes brought by this progress in the understanding, diagnosis, and management of these disorders. Application of these methods makes it possible to reach an early diagnosis, therefore allowing early treatment, which has led to a profound improvement in outcomes when compared to previous management.

Low-grade "benign" birdshot retinochoroiditis: prevalence and characteristics.

PURPOSE: To establish the prevalence, morphologic and functional characteristics and evolution of mild birdshot retinochoroiditis (BRC). METHODS: Retrospective review of all BRC cases treated at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland, with at least 3 years of follow-up since the initial symptoms. Sub-Tenon's injection of triamcinolone was the first line of treatment if visual field changes were unilateral, with no additional treatment if visual field returned to normal. The percentage of patients who did not need systemic therapy was established, and the following parameters were evaluated: demographic characteristics, best-corrected visual acuity (BCVA), fundus photographs, fluorescein angiography (FA) and indocyanine green (ICGA) angiography frames and perimetry evaluation from initial visit to last follow-up. RESULTS: Twenty cases of BRC were included in this study. Three of these patients (15%) received only local periocular therapy and qualified as mild BRC, with a mean follow-up of 9.3 years. The BCVA was 0.89 ± 0.25 at presentation and 1.0 ± 0.39 at last follow-up. Average visual field mean defect was 5.05 ± 3.27 at presentation and 1.78 ± 0.95 at last follow-up. Depigmented fundus lesions remained stable from presentation to last follow-up. Choroidal inflammatory activity monitored by ICGA decreased from 11.66 ± 3.44 at presentation to 4.25 ± 2.87 at last follow-up. FA revealed mild retinal vasculitis (2.25 ± 3.20) which remained stable (2.00 ± 4.00). CONCLUSIONS: In our setting, 15% of BRC cases had a benign course, controlled with periocular treatment. It is important to identify such cases in order not to overtreat, exposing patients needlessly to potential side effects of aggressive and prolonged immunosuppressive treatment.

Catching the therapeutic window of opportunity in early initial-onset Vogt-Koyanagi-Harada uveitis can cure the disease.

PURPOSE: Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune granulomatous choroiditis that begins in the choroidal stroma. The aim of this review was to gather a body of evidence for the concept of a window of therapeutic opportunity, defined as a time interval following initial-onset disease during which adequate treatment will substantially modify the disease outcome and possibly even lead to cure, similar to what has been described for rheumatoid arthritis. METHODS: We reviewed the literature and consulted leading experts in VKH disease to determine the consensus for the notion of a therapeutic window of opportunity in VKH disease. RESULTS: We found a substantial body of evidence in the literature that a therapeutic window of opportunity exists for initial-onset acute uveitis associated with VKH disease. The disease outcome can be substantially improved if dual systemic steroidal and non-steroidal immunosuppressants are given within 2-3 weeks of the onset of initial VKH disease, avoiding evolution to chronic disease and development of "sunset glow fundus." Several studies additionally report series in which the disease could be cured, using such an approach. CONCLUSIONS: There is substantial evidence for a therapeutic window of opportunity in initial-onset acute VKH disease. Timely and adequate treatment led to substantial improvement of disease outcome and prevented chronic evolution and "sunset glow fundus," and very early treatment led to the cure after discontinuation of therapy in several series, likely due to the fact that the choroid is the sole origin of inflammation in VKH disease.

QUANTITATIVE ANALYSIS OF STRUCTURAL ALTERATIONS IN THE CHOROID OF PATIENTS WITH ACTIVE BEHÇET UVEITIS.

PURPOSE: To quantitatively analyze in vivo morphology of subfoveal choroid during an acute attack of Behçet uveitis. METHODS: In this prospective study, 28 patients with Behçet uveitis of ≤4-year duration, and 28 control subjects underwent enhanced depth imaging optical coherence tomography. A novel custom software was used to calculate choroidal stroma-to-choroidal vessel lumen ratio. Subfoveal choroidal thickness was measured at fovea and 750 µm nasal, temporal, superior, and inferior to fovea. Patients underwent fluorescein angiography and indocyanine green angiography. Receiver operating characteristic curve and area under the curve were computed for central foveal thickness. The eye with a higher Behçet disease ocular attack score 24 was studied. The main outcome measures were choroidal stroma-to-choroidal vessel lumen ratio and choroidal thickness. RESULTS: The mean total Behçet disease ocular attack score 24, fluorescein angiography, and indocyanine green angiography scores were 7.42 ± 4.10, 17.42 ± 6.03, and 0.66 ± 0.73, respectively. Choroidal stroma-to-choroidal vessel lumen ratio was significantly higher in patients (0.413 ± 0.056 vs. 0.351 ± 0.063, P = 0.003). There were no significant differences in subfoveal choroidal thickness between patients and control subjects. Choroidal stroma-to-choroidal vessel lumen ratio correlated with retinal vascular staining and leakage score of fluorescein angiography (r = 0.300, P = 0.036). Central foveal thickness was significantly increased in patients (352.750 ± 107.134 µm vs. 263.500 ± 20.819 µm, P < 0.001). Central foveal thickness showed significant correlations with logarithm of minimum angle of resolution vision, Behçet disease ocular attack score 24, total fluorescein angiography score, retinal vascular staining and/or leakage and capillary leakage scores of fluorescein angiography, and total indocyanine green angiography score. At 275 µm cutoff, diagnostic sensitivity and specificity of central foveal thickness for acute Behçet uveitis were 89% and 72%, respectively (area under the curve = 0.902; 95% CI = 0.826-0.978, P < 0.001). CONCLUSION: There was choroidal stromal expansion which was not associated with thickening of the choroid. Central foveal thickness may be used as a noninvasive measure to assess inflammatory activity in early Behçet uveitis.

Need for Quantitative Measurement Methods for Posterior Uveitis: Comparison of Dual FA/ICGA Angiography, EDI-OCT Choroidal Thickness and SUN Vitreous Haze Evaluation in Stromal Choroiditis.

BACKGROUND/PURPOSE: Quantitative methods for posterior uveitis are necessary for precise appraisal and follow-up of inflammation in practice and in clinical trials. The aim of this study was to assess fluorescein angiography (FA), indocanine green angiography (ICGA), and enhanced depth imaging optical coherence tomography choroidal thickness (EDI-OCT CT) in two stromal choroiditis entities, birdshot retinochoroiditis (BRC), and Vogt-Koyanagi-Harada disease (VKH), as well as to determine (1) disease patterns, (2) respective response to therapy, and (3) their potential utility in clinical trials in comparison to vitreous haze, the present standard outcome used in clinical trials. METHODS: This retrospective study included newly diagnosed patients with BRC and VKH, seen at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiographic signs were quantified using an established dual FA/ICGA scoring system for uveitis at presentation and on follow-up. FA/ICGA score ratios were compared between diseases to determine disease patterns. EDI-OCT CT was determined using a spectral domain instrument. Vitreous haze was determined using the SUN (Standardization of Uveitis Nomenclature) method. RESULTS: Among 1872 uveitis patients seen from 1995 to 2016, 8 newly diagnosed BRC patients (16 eyes) and 6 newly diagnosed VKH patients (12 eyes) had sufficient data for study inclusion. Patients with BRC and VKH at initial onset had mean FA scores of 16.1 ± 7.0 vs. 4.6 ± 2.1 (p < 0.0001), respectively, while mean ICGA scores were similarly high in the two diseases, 18.9 ± 3.6 (BRC) vs. 20.8 ± 7.5 (VKH). After therapy, FA and ICGA scores decreased significantly for both entities (- 60% of FA score and 55% of ICGA score in BRC vs. - 72% of FA score and - 87% for ICGA score in VKH). EDI-OCT CT decreased significantly in the two entities. Vitreous haze was almost absent in VKH and low in BRC. CONCLUSION: Dual FA/ICGA scoring showed the diverse disease patterns of BRC and VKH; both the retina and choroid were involved at onset in BRC, whereas VKH was a pure choroidal disease with later spillover into the retina. Dual FA/ICGA allowed for the precise measurement of inflammation at onset and upon follow-up. EDI-OCT CT responded to therapy in both diseases but was found to be of limited use in this early/subacute disease phase because it lacked sensitivity to detect subclinical recurrences and was therefore only useful for long-term follow-up. Vitreous haze was low in both entities and thus useless as an inflammatory parameter.

Contribution of dual fluorescein and indocyanine green angiography to the appraisal of posterior involvement in birdshot retinochoroiditis and Vogt-Koyanagi-Harada disease.

PURPOSE: To assess the levels of retinal and choroidal involvement in initial-onset birdshot retinochoroiditis (BRC) and Vogt-Koyanagi-Harada (VKH) disease, two stromal choroiditis entities. METHODS: This retrospective study included patients diagnosed with BRC and VKH, seen during initial-onset disease at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiographic signs were quantified, using an established dual fluorescein angiography (FA) and indocyanine green angiography (ICGA) scoring system for uveitis, and the FA/ICGA score ratios were compared between diseases. RESULTS: Among 1793 patients with uveitis seen from 1995 to 2015, 7 newly diagnosed BRC patients and 4 patients with newly diagnosed VKH disease had sufficient data for study inclusion. Patients with BRC and VKH at initial onset had mean FA angiographic scores of 16.91 ± 3.42 and 4.06 ± 1.87; mean ICGA angiographic scores of 21.34 ± 3.49 and 25.75 ± 3.88; and mean FA/ICGA ratios of 0.79 ± 0.21 and 0.16 ± 0.09, respectively. CONCLUSION: This study showed the differential involvements of the retina and choroid in BRC and VKH. The choroid was preponderantly involved in both diseases; thus, ICGA is essential for disease assessment and follow-up. However, these diseases also differed substantially. The origin of inflammation was primarily in the choroid in VKH and in both the choroid and retina in BRC. We recommend dual FA and ICGA for evaluating posterior uveitis, when choroiditis is suspected.

The importance of quantitative measurement methods for uveitis: laser flare photometry endorsed in Europe while neglected in Japan where the technology measuring quantitatively intraocular inflammation was developed.

Laser flare photometry (LFP) is an objective and quantitative method to measure intraocular inflammation. The LFP technology was developed in Japan and has been commercially available since 1990. The aim of this work was to review the application of LFP in uveitis practice in Europe compared to Japan where the technology was born. We reviewed PubMed articles published on LFP and uveitis. Although LFP has been largely integrated in routine uveitis practice in Europe, it has been comparatively neglected in Japan and still has not received FDA approval in the USA. As LFP is the only method that provides a precise measure of intraocular inflammation, it should be used as a gold standard in uveitis centres worldwide.

Enhanced depth imaging is less suited than indocyanine green angiography for close monitoring of primary stromal choroiditis: a pilot report.

The purpose of this study is to investigate the performance, utility, and precision of enhanced depth imaging optical coherence tomography (EDI-OCT) versus indocyanine green angiography (ICGA) in tracking any fluctuation in the activity of stromal choroiditis in response to therapeutic interventions during long-term follow-up. Patients with a diagnosis of Vogt-Koyanagi-Harada (VKH) disease or birdshot retinochoroiditis (BRC), with untreated initial disease, and having had long-term follow-up, including both ICGA and EDI-OCT, were recruited at the Centre for Ophthalmic Specialised care, Lausanne, Switzerland. Angiography signs were quantified according to established dual fluorescein angiography (FA) and ICGA scoring systems for uveitis. Changes in ICGA score and EDI choroidal thickness, in response to therapeutic intervention, were assessed. In the four eyes analysed (2 BRC and 2 VKH), mean EDI-OCT choroidal thickness decreased from 672 ± 101 µm at presentation to 358.5 ± 44.5 µm in a mean of 26.5 months, i.e. the time taken to stabilize the disease. Mean ICGA scores decreased from 28 ± 4.2 at presentation to 5 ± 7 at stabilization. Only ICGA was sufficiently sensitive and reactive having the ability to detect disease recurrences and efficacy or the absence of effect of successive treatment changes, detected in seven instances during follow-up, not recorded by EDI-OCT. This pilot study showed that ICGA was a more sensitive methodology, which promptly identifies evolving subclinical and occult choroidal disease, and flag occult recurrence and/or therapeutic responses that were otherwise missed by EDI-OCT. Although choroidal thickness was proportional to treatment course, demonstrating a linear decrease, these changes were too sluggish to be relied upon for close follow-up and timely adjustment of therapy.

Reappraisal of the management of Vogt-Koyanagi-Harada disease: sunset glow fundus is no more a fatality.

PURPOSE: Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune stromal choroiditis. Aim of the study was to gather a body of evidence from the literature and from experts that systemic corticosteroid combined with non-steroidal immunosuppressive therapy should become the standard of care in initial-onset VKH disease. METHODS: Literature was reviewed and leading experts in VKH were consulted in different parts of the world in order to put forward a consensus attitude in the management of initial-onset VKH disease. RESULTS: There was a substantial body of evidence in the literature that early aggressive and sustained corticosteroid and non-steroidal immunosuppressive therapy in initial-onset VKH disease allows to achieve full control of choroidal inflammation, eliminating any subclinical choroidal inflammation, and substantially reduces recurrences with improvement of anatomical and functional outcomes. This was in agreement with experts' opinion and practice. ICGA was the method of choice to monitor disease evolution. CONCLUSION: Since the choroidal space is easily accessible to systemic therapy and because inflammation in VKH disease is exclusively originating from the choroidal stroma, early and sustained treatment right at the onset of the disease process with dual corticosteroid and non-steroidal immunosuppressive therapy can result in full "healing" in many cases preventing sunset glow fundus which results from depigmentation from chronic uncontrolled inflammation.

Interpretation of fundus autofluorescence changes in choriocapillaritis: a multi-modality imaging study.

PURPOSE: We aimed to describe imaging findings in primary inflammatory choriocapillaropathies (PICCPs) after a photobleaching process. METHODS: 2Images from six consecutive cases of patients affected by PICCPs (four with multiple evanescent white dot syndrome and two with multifocal choroiditis) were reviewed. Patients underwent fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral-domain optical coherence tomography (SD-OCT) by means of the Spectralis HRA (Heidelberg Engineering, Heidelberg, Germany). FAF images were acquired at the beginning of the examination in partially dark-adapted conditions followed by light adapted conditions. RESULTS: During the active phase of the diseases, all patients showed areas of increased FAF that became isoautofluorescent after photobleaching. Simultaneously with increased FAF, the ICGA showed typical hypofluorescent dark areas that were more evident in the late phase. SD-OCT showed disruptions in the hyper-reflective band at the ellipsoid zone. CONCLUSIONS: FAF and SD-OCT are complementary imaging techniques that show alterations in the outer retina of patients affected by PICCPs.

Fulminant proliferative diabetic retinopathy in the non-photocoagulated eye following acute renal failure.

Management of diabetic retinopathy should follow more strict and aggressive rules in patients at risk for severe acute renal impairment. Such patients should be identified and possibly prophylactically laser treated to avoid the severe consequences demonstrated in this case report. A 34-year-old type 2 diabetes patient with a stabilized diabetic retinopathy developed acute and severe retinal decompensation within weeks after acute renal failure complicated his chronic stable renal impairment. Fluorescein angiographic and optical coherence tomographic illustrations of the rapid evolution of the retinal condition are presented. The patient had previously been treated with panretinal photocoagulation in his left eye. After 8 years of regular 6-monthly checked stability, he developed rapid-onset proliferative diabetic retinopathy and macular edema in his right eye within 3 months of his last ocular check-up. Fluorescein angiography showed neovessels and major ischemic areas. Emergency panretinal photocoagulation and a sub-Tenon's injection were necessary to achieve control of the situation with regression of neovessels and complete regression of macular edema. This case shows that it is imperative for nephrologists to be well informed about a patient's ocular situation in order to give timely information to the ophthalmologist who can intervene to protect the retina in case of renal failure. On the other hand, the ophthalmologist should be familiar with the renal function of his patient with renal impairment so that he can decide to perform prophylactic retinal panphotocoagulation that should be imperatively considered even without strict indications in patients with renal impairment at risk for further deterioration of renal function, in order to prevent such explosive ischemic and proliferative retinopathy putting vision at risk.

New concepts in the appraisal and management of birdshot retinochoroiditis, a global perspective.

Birdshot retinochoroiditis (BRC) is a relatively recently described entity. BRC uveitis is predominant in the posterior segment with dual, independent retinal, and choroidal inflammation. The disease has no known extra-ocular inflammation sites and yet features the strongest known HLA association: HLA-A29 is present in close to 100 % of cases. Aim in this mini-review was to readjust the appraisal of BRC in the light of a global approach including the full array of investigational procedures. Historical background and the genesis of the disease name were searched. Global disease description including both the retina and the choroid was given. Retinal involvement was clearly characterized, pointing toward the profuse leakage of retinal vessels of all sizes in early disease and widespread atrophy in under treated patients. The importance of exploration of choroidal disease, unavailable until the early 1990s before the advent of indocyanine green angiography (ICGA) was stressed, allowing early diagnosis of disease. Despite its proven importance to explore the choroid, ICGA is still sparsely used. Existing diagnostic criteria were found to be clearly inappropriate not allowing early diagnosis and are in need to be revised, taking into account both retinal and choroidal aspects of the disease, in order to make early diagnosis possible and hence allow proper management .

Early and sustained treatment modifies the phenotype of birdshot retinochoroiditis.

In this single-centre retrospective case review, we investigate the long-term follow-up of birdshot retinochoroiditis (BRC) patients, analysing the impact of early, vigorous, and prolonged treatment on the evolution of indocyanine green angiography (ICGA) signs and fundus appearance. Treatment delay was calculated for each BRC patient, and patients were classified into two groups--treatment delay of <10 months (early-treatment group) and treatment delay of >10 months (delayed-treatment group). Fundus photographs and ICGA frames from the initial visit and from the last follow-up visit were assessed. Fundus photographs were evaluated for the presence of at least three circumpapillary, typical, rice-shaped birdshot lesions in one eye, inferior or nasal to the optic disc. ICGA pictures were evaluated for the presence of lesions (hypofluorescent dark dots, fuzziness). Differences were compared between the two groups and between the first visit and the last follow-up visit. In the early-treatment group, 5/6 patients had no characteristic BRC fundus lesions, but 7/7 patients in the delayed-treatment group displayed typical lesions. At last follow-up, 5/6 early-treatment patients showed no fundus lesions, and 6/7 delayed-treatment patients retained their fundus lesions. At presentation, all 13 patients exhibited lesions on ICGA. At last follow-up, ICGA lesions had completely disappeared in 4/6 early-treatment patients and 3/7 delayed-treatment patients. Thus, early and sufficiently dosed inflammation-suppressive treatment can prevent the appearance of typical BRC fundus lesions. It is therefore crucial to perform ICGA to detect otherwise occult stromal choroiditis in suspected BRC cases and to initiate adequate therapy immediately.