Demyelinating plaque-associated uveitis.

PURPOSE: To report the clinical and fluorescein angiographic (FA) features of demyelinating plaque-associated uveitis (DPU), a subset of uveitis in which patients have demyelinating plaques on the brain/cervical magnetic resonance image (MRI) but do not meet the criteria for multiple sclerosis (MS). METHODS: In this retrospective observational study, Persian Patients were diagnosed with DPU and included if (1) they never satisfied the MS criteria, (2) all other possible etiologies were excluded, and (3) they were followed for at least 2 years. RESULTS: After a median follow-up of 3 years (interquartile range, 2.0-5.3), 8 out of 40 (20%) patients diagnosed with DPU were excluded as they subsequently met the MS criteria. Of remaining 32 patients studied, the mean age was 36.3±9.9 (range 20-56 years), and 30 (93.8%) were female. Twenty-four (75.0%) showed bilateral involvement and 27 (84.4%) had insidious-chronic course. Uveitis was classified as intermediate (with or without anterior uveitis) in 29 (90.6%) and isolated anterior in 3 (9.4%) patients. Nine (28.1%) patients had at least one systemic neurological complaint. Ocular findings were: granulomatous keratic precipitates in 43/44 (97.7%) eyes; snowballs in 25/52 (48.1%) eyes; snowbanks in 4/52 (7.7%) eyes; cystoid macular edema in 20/56 (35.7%) eyes; and optic neuritis in 5/56 (8.9%) eyes. Visual acuity was ≥ 20/40 in 39 eyes (69.6%) at presentation which improved to 46 eyes (81.2%) at 2-year follow up. The two most frequent findings in FA were optic disc leakage/staining in 44/52 (81.5%) eyes, and peripheral retinal perivascular leakage in 39/52 (76.9%) eyes, which in 14/52 (26.9%) eyes extended beyond the equator. CONCLUSION: DPU usually presents as a bilateral chronic granulomatous intermediate and, less often, isolated anterior uveitis, especially in females. Most are neurologically asymptomatic. Visual outcome is generally favorable. In FA, peripheral retinal perivascular leakage is common. DPU patients have an increased tendency to develop MS and should be prohibited from anti-TNF treatment.

Assessment of measurement methods of posterior inflammation in stromal choroiditis: the value of quantitative outcome measures versus the presently qualitatively based paradigm.

PURPOSE: To assess posterior inflammation using a fluorescein (FA)/indocyanine-green angiography (ICGA) scoring system, and compare them to the presently recommended outcome measure, the standardization of uveitis nomenclature vitreous haze score (SUN-VH) in stromal choroiditis. METHODS: This was a retrospective study on patients with a diagnosis of ocular sarcoidosis(OS), ocular tuberculosis(OT), Birdshot retinochoroiditis(BRC) and Vogt-Koyanagi-Harada disease(VKH) seen in the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiography signs were quantified according to an established FA/ICGA scoring system. Vitritis was assessed using SUN-VH. Results were compared. RESULTS: 65 newly diagnosed patients (128 eyes) with stromal choroiditis were included. Angiographic scoring showed variable degrees of choroidal versus retinal involvement (87% for OS, 72% for OT, 62.5% for BRC and 100% for VKH). On the other hand, a mere 22 of 128 eyes (17%) showed a SUN-VH score ≥ 2 necessary for inclusion in clinical trials. Moreover, FA/ICGA values followed a normal distribution curve and presented inter-examiner variations greater than 1-SD in only 8.4% of cases. SUN-VH values' distribution was non-normal and showed inter-examiner discrepancies greater than 1-SD in 51.7% of cases. CONCLUSION: This study highlights the precise measurement of global posterior inflammation achieved by a dual FA/ICGA scoring system in stromal choroiditis. In contrast, SUN-VH scale appears imprecise and inadequate, as only a minute percentage of the studied eyes could have been included in a clinical trial based on this criterion. To evaluate posterior intraocular inflammation meaningfully in stromal choroiditis, the use of dual FA/ICGA is strongly advised and should replace the presently recommended SUN-VH system.

Torpedo Maculopathy Associated with Choroidal Neovascularization.

Background Torpedo maculopathy is a very rare, congenital, usually unilateral hypopigmented lesion in the temporal macula. Material and Methods This retrospective case series describes three patients with torpedo maculopathy. Results The first two cases demonstrate typical clinical and imaging findings of torpedo maculopathy in asymptomatic patients. The third case relates to a symptomatic young patient with a torpedo lesion, a smaller satellite lesion, and evidence of choroidal neovascularization confirmed by fluorescence angiography. In the area of the clinically visible torpedo lesion, spectral domain optical coherence tomography showed atrophy of the outer retina with increased choroidal signalling and a hyperreflective lesion above the retinal pigment epithelium suggestive of choroidal neovascularization. Fundus autofluorescence imaging revealed a hyperautofluorescent rim along the margin of the hypoautofluorescent torpedo lesion. Conclusion In the literature, torpedo lesions are usually regarded as benign lesions with no tendency for progression. The third case demonstrates that torpedo lesions may be associated with choroidal neovascularization, which has been successfully treated with anti-VEGF therapy.

Evolution of choroidal thickness over time and effect of early and sustained therapy in birdshot retinochoroiditis.

PurposeTo follow choroidal thickness (ChT) over time in birdshot retinochoroiditis (BRC) using enhanced depth imaging optical coherence tomography (EDI-OCT) and study the effect of early and sustained treatment on ChT.Patients and methodsEighteen patients were included and EDI-OCT measurements of ChT were analyzed retrospectively in five groups of patients with follow-up times ranging from 1 year to ≥15 years. The OCT images were evaluated and ChT was calculated under the foveola and 1500 µm temporal, nasal, superior, and inferior to the foveola. To assess the effect of treatment, 13 patients with a disease duration ≥10 years were divided into two groups depending on their treatment status: early and sustained therapy vs insufficient, late, or no treatment. ChT was compared in these two groups along with the number of typical fundus BRC lesions.ResultsThe ChT decreased (r=-0.41, P=0.0018) over the disease duration, which ranged from <1 year to ≥15 years. In patients with a disease duration ≥10 years, a significant difference in ChT was noted between adequately and undertreated patients (288.3±76.9 µm vs 161.4±39.2 µm; P=0.004). At the last follow-up, in the group with insufficient therapy 10 of 11 eyes presented typical fundus BRC lesions vs 2 of 13 eyes in the treated group (P≤0.0006, F-test).ConclusionsChoroidal thickness decreases significantly over time in BRC. If undertreated, patients show thinner choroids compared with adequately treated individuals and present significantly more BRC lesions.

SLO Fundus Imaging Is the Most Sensitive Modality of Multimodal Imaging for Macular Microembolisms with Subtle Signs.

Background. Microemboli of fat or other material into the terminal macular retinal circulation can be difficult to diagnose. We report 2 cases that showed subtle signs where SLO fundus imaging was most sensitive to precisely outline the limits of the inner retina infarction. Patients and Methods. Multimodal imaging analysis was performed including fundus photography, fluorescein angiography, indocyanine green angiography, Optical Coherence Tomography and SLO fundus imaging of 2 cases with suspected infarction of the inner retina. Cases. A 30-year-old man reported a grey central spot OD a few days after being squeezed between two cars with a sacrum fracture. Vision was 0.2 OD, and 1.0 OS. Examination was unremarkable and fluorescein angiography was normal. Octopus visual field showed a tiny central scotoma OD. Microperimetry showed decreased central sensitivity OD > OS. The only sign was a dark area on the SLO fundus picture indicating a subtle infarction of the inner retina (OD > OS) with nothing visible on the OCT. Resolution of lesions on the SLO picture ODS occurred in parallel with improvement of microperimetry and visual acuity. A 32-year-old woman suspected to take IV drugs had a sudden drop of vision to 0.4 OD and count fingers at 6 feet OS. Signs included macular hemorrhages and non perfusion on FA. The striking sign was a large dark area on the SLO picture precisely delineating the more extensive infarcted area of internal retina corresponding to OCT hyperreflectivity, visible in this case. Conclusions. Macular ischemia due to microemboli can show obvious fundus signs as hemorrhages, cotton wool spots and non perfusion or can present in a subclinical fashion. The SLO picture has a higher image contrast and higher resolution compared to conventional fundus photography and so can precisely delineate ischemic changes of the inner retina causing the unexplained visual loss.

Discrepancy between Visual Acuity and Microperimetry in AMD Patients: Visual Acuity Appears as an Inadequate Parameter to Test Macular Function.

BACKGROUND: Best corrected visual acuity (BCVA) of 0.8 or above in AMD patients can sometimes correspond to poor macular function inducing a serious visual handicap. Microperimetry can be used to objectivize this difference. PATIENTS AND METHODS: A retrospective study was undertaken on 233 files of AMD patients of whom 82 had had a microperimetry. BCVA was compared with microperimetry performance. All examinations were performed in an identical setting by the same team of 3 persons. RESULTS: Among the 82 patients included, 32 (39.0%) had a BCVA equal to or above 0.8 even though their microperimetry performance was lower than 200/560 db. 10 of them (12.2% of total) had an even poorer microperimetry below 120/560 db indicating poor macular function. CONCLUSIONS: More than a third of the AMD patients had a bad or very bad microperimetry performance in parallel with a good visual acuity. Microperimetry is a valuable tool to assess and follow real macular function in AMD patients when visual acuity alone can be misleading.

Psoriasis beyond the skin surface: a pilot study on the ocular involvement.

The ocular involvement in psoriasis is not a completely well-known problem. The ophthalmologic involvement occurs in about 10 % of patients, particularly in case of arthropathic or pustular psoriasis. Ocular lesions are more common in males, and they often occur during psoriasis exacerbations. Our study aimed to assess the prevalence and type of ocular involvement in psoriasis, by a comparison between psoriasis and healthy subjects, and if/how a 12-week long systemic immunosuppressive therapy is able to modify them. This study involved thirty-two psoriatic patients and thirty-two healthy subjects. Dermatological evaluation was done using Psoriasis Area and Severity Index, Physician Global Assessment, and Dermatology Life Quality Index (PASI, PGA, and DLQI score). Ophthalmological evaluation included ocular surface involvement (Schirmer, Jones, break-up time--BUT, DR-1 camera), retinal pathologies, and ocular surface disease index. Laboratory investigations including the C-reactive protein (CRP) of all the patients were performed. At baseline, the values of Schirmer, Jones, and BUT tests in the patient group were significantly lower compared to controls; moreover, conjunctival hyperemia was more frequent in psoriatic patients than in healthy subjects. Ocular involvement was more prominent in the subset of psoriatic patients with sebo-psoriasis than in general psoriatic population. A statistically significant correlation was found in sebo-psoriasis between PASI and Schirmer, between PASI and Jones, and between PASI and BUT. On the other hand, the results obtained from DR1 camera showed statistically significant difference between psoriatic and sebo-psoriatic patients at the end of the follow-up. After 12 weeks of treatment, the mean values of PASI, PGA, DLQI, CRP, and BUT showed significant changes in psoriatic patients. Our findings suggest a high rate of ocular involvement in psoriatic patients, emphasizing the need of performing periodic ophthalmological examinations in order to avoid underestimating eye diseases and to allow early diagnosis and treatment of patients.

Classification of choroiditis based on inflammatory lesion process rather than fundus appearance: enhanced comprehension through the ICGA concepts of the iceberg and jellyfish effects.

Choroidal inflammatory diseases have been classically grouped under the term of white dot syndromes (WDS), a term only based on the appearance (white-yellow dots) of inflammatory fundus lesions. This purely descriptive and vague terminology, regrouping a pot-pourri of posterior inflammatory conditions, probably came into use because the precise exploration of the choroid was not possible, and also because many of the diseases were rare and not well understood. Since the availability of indocyanine green angiography (ICGA) that allows one to explore the choroidal compartment, it became possible to understand the lesion mechanism of choroiditides and to classify this group of diseases according to their pathophysiological behaviour. It was our aim to show here that the term WDS is applied to and encompasses inflammatory conditions that are characterized by completely different lesion mechanisms and should therefore be classified separately from each other. ICGA made it possible to differentiate two types of choroiditides, including on the one hand inflammatory diseases of the choroidal stroma and on the other hand inflammatory diseases of the choriocapillaris. Unfortunately, twenty years after its advent, ICGA is still not used routinely in uveitis centres and the traditional inappropriate but overall useless term of WDS is still used, maintaining the confusion about these diseases. The aim of this work was (i) to illustrate that meaningful exploration of choroidal inflammation, mostly occult and inaccessible to usual investigations, has to be performed using ICGA, (ii) to insist on the crucial importance of ICGA in the management of choroiditis and (iii) to enhance the comprehension of the ICGA-based classification of choroiditis, by using the demonstrative and striking analogue concepts of iceberg and jellyfish effects.

Indocyanine green angiography (ICGA) is essential for the early diagnosis of birdshot chorioretinopathy.

BACKGROUND: Birdshot chorioretinitis (BC) is a rare disease involving the retina and the choroid independently. The hallmark for BC is the presence of depigmented oval lesion of the choroid, the so called "birdshot lesions", however in the early phase of disease these lesions are often not visible. METHODS: A retrospective analysis of BC patients that were investigated in Centre for Ophthalmic Specialised Care, Lausanne, Switzerland between 1995 and 2010 was performed. Patients seen in the initial phase of BC disease devoid of a specific diagnosis when referred were included. Clinical investigations along with fluorescein angiography (FA), indocyanine green angiography (ICGA) and visual field testing (VF) were analysed. RESULTS: Three out of 7 patients (43 %) seen in the initial phase of the disease devoid of a diagnosis at presentation were analysed. These patients presented with no "birdshot" lesions whatsoever. All three patients were HLA-A29 positive, presented with vitreitis and retinal vasculitis on FA. On ICGA, all 3 patients presented bilateral evenly distributed choroidal hypofluorescent dark dots (HDD) representing choroidal granulomas. CONCLUSIONS: ICGA, by providing occult information on the choroid, is an essential tool for early diagnosis of BC. Because ICGA is still not universally practiced in uveitis centres early disease is often missed, its diagnosis delayed and proper treatment started late.

["White dot syndromes" in childhood]. / "White-dot-Syndrome" im Kindesalter.

BACKGROUND: The term "white dot syndromes" describes a group of heterogeneous inflammatory disorders of the choriocapillaris. They were first described a few decades ago and our knowledge about these variable diseases is very limited, especially in regard to their overall incidence and their differential diagnostic relevance in uveitis of childhood. MATERIAL AND METHODS: A retrospective analysis has been performed of all cases of white dot syndromes in 407 patients with paediatric uveitis who were examined between 1996 and 2006. The relevant literature was reviewed. RESULTS: The following incidence of white dot syndromes in childhood was found: acute posterior multifocal placoid pigmentepitheliopathy (APMPPE) (n = 4), multiple evanescent white dot syndrome (MEWDS) (n = 4), multifocal choroiditis and panuveitis (MCP) (n = 3) and 1 case of serpiginous choroiretinitis. The review of literature shows a different age predilection of the different white dot diseases. The relative frequency of white dot syndromes in paediatric uveitis patients is estimated to be between 1 - 5%. CONCLUSION: "White dot syndromes" are an important differential diagnosis in uveitis of childhood. ICG angiography is an important tool for the diagnosis and follow-up examinations of these inflammatory diseases of the choriocapillaris. The different entities of white dot syndromes show differences concerning incidence, prevalence, course of disease, rate of complications and therapeutic implications.

Deep sclerectomy for the management of uncontrolled uveitic glaucoma: preliminary data.

BACKGROUND: If medical treatment fails in uveitic glaucoma a surgical approach should be considered. Classical trabeculectomy is known to have a less favourable outcome in uveitis. Our intention is to report the first series of uveitis patients with glaucoma resistant to medical therapy who were treated with deep sclerectomy (DS). PATIENTS AND METHODS: Fourteen eyes of 13 patients (mean age 39.0 +/- 18.5 years; range 8 to 76 years) with chronic uveitis underwent non-penetrating filtering surgery from 1995 to 2003. All patients had their uveitis controlled before and after surgery by immunomodulatory therapy. Non-penetrating filtering surgery consisted of DS with collagen implant (Staar(R)) in 4 eyes, DS with draining device (T-Flux Ioltech(R)) in 2 patients, DS without implant in 7 patients and with viscocanalostomy in 1 patient. Nine eyes (65 %) received mitomycin C peri-operatively. RESULTS: Intra-ocular pressure (IOP) was reduced from a mean pre-operative value of 42.8 +/- 13.6 mmHg to a 1-year mean post-operative value of 12.1 +/- 4.0 (71.7 % reduction). Eleven of the 14 eyes completed 12 months of follow-up, resulting in complete success in 5 (45.4 %) and in qualified success in 5 (45 %) and in failure in one patient (9.2 %), later controlled by a second operation. Anti-glaucomatous medication was reduced from a mean of 3.7 +/- 0.5 medications preoperatively to 1.2 +/- 0.8 medications (71.4 % reduction) at the 12 month follow-up. Nine of the 14 patients achieved a 24 month follow-up with a mean IOP of 14.1 +/- 3.8 mmHg and mean of anti-glaucomatous medications of 1.6. Four patients have been examined 4 years after the DS: mean IOP was 13.2 +/- 2.2 mmHg and mean medication 1.7 +/- 1.0. Post-operative complications included one case of lens opacity and 2 cases of hypotony lasting for five months and four weeks after the intervention respectively. CONCLUSION: Non-penetrating filtering surgery controlled the intra-ocular pressure in 90 % of eyes with uveitic glaucoma resistant to medical therapy at 12 months. Surgical complications were low which may explain the high success rate of the procedure, compared to classical penetrating surgery.

Differential inflammatory involvement in retina and choroïd in birdshot chorioretinopathy.

BACKGROUND: Birdshot chorioretinopathy is characterised by dual unrelated inflammatory involvement of the retina and the choroid. Indocyanine green angiography made it possible to assess and follow choroidal disease with the same precision as retinal involvement was followed so far. The aim of this study was to analyse the severity, progression and response to therapy of both retinal involvement using fluorescein angiography and choroidal involvement using indocyanine green angiography. PATIENTS AND METHODS: Patients with birdshot retinochoroidopathy followed at La Source Eye Centre in Lausanne, Switzerland from January 1995 to December 2002 were subdivided into three subgroups according to the duration of evolution of the disease: untreated patients with no more than one year duration of the disease (group 1, n = 6); treated patients with disease duration of 1 - 7 years duration (group 2, n = 5) and patients with disease lasting for more than 7 years (group 3, n = 4). Fluorescein and indocyanine green angiographic signs (angiographic scores given by a masked observer) were analysed in the 3 groups and compared to the "cream-coloured" fundus lesions. RESULTS: Fifteen out of the 742 patients (2.0 %) seen at La Source Eye Centre during the time period considered presented BC and were included in the study. In the "early disease group" fluorescein and ICG angiography showed more severe choroidal than retinal involvement with respective scores of 3 +/- 0.79 (ICG) and 2 +/- 1.17 (FA) while there were few depigmented fundus lesions to be seen (score 1 +/- 0.27). The choroidal involvement responded well to systemic corticosteroids +/- immunosuppressive therapy (scores in groups 2 and 3 = 1.2 and 0.75), while retinal disease was stabilised at best (scores in groups 2 and 3 = 2.2. and 2.4) and depigmented fundus lesions increased (scores in groups 2 and 3 = 2.8 and 3). CONCLUSION: The evolution and response to therapy of retinal and choroidal disease in birdshot chorioretinopathy have a different course with choroidal disease responding well to therapy while retinal disease is more resistant, possibly explaining the slow deterioration of functional parameters despite therapy. The increase of "cream-coloured" fundus lesions despite good choroidal response to therapy could be explained by depigmentation left behind after resolution of choroidal stromal granulomas, a hypothesis recently confirmed by an autopsy case of birdshot chorioretinopathy.

Detection of Toxoplasma gondii DNA in primary intraocular B-cell lymphoma.

Primary intraocular lymphoma, a variant of primary central nervous system lymphoma with ocular involvement, is a large B-cell non-Hodgkin's lymphoma. Some cases of primary intraocular lymphoma have been reported to be associated with microorganisms including Epstein-Barr virus (EBV) and human herpes virus-8 (HHV-8), but not parasites. We analyzed 10 cases of primary intraocular lymphoma using microdissection and PCR. Tumor and normal cells were microdissected from ocular tissue on slides and subjected to PCR for genes from Toxoplasma gondii, EBV, and HHV-8. We detected Toxoplasma gondii, not HHV-8 or EBV, DNA in the lymphoma but not in normal cells of two cases that resembled ocular toxoplasmosis clinically. We speculate that Toxoplasma gondii may play a role in some forms of primary intraocular B-cell lymphoma.

Value of high-frequency ultrasound biomicroscopy in uveitis.

PURPOSE: To determine the use of high-frequency ultrasound biomicroscopy (UBM) in the assessment of inflammatory lesions of the iris, ciliary body, pars plana and peripheral vitreous, and in particular to determine the proportion of cases for which UBM contributed significant additional, hitherto inaccessible, information. METHODS: Charts of patients seen in the uveitis clinic at University Eye Hospital from November 1994 to September 1999 for whom a UBM investigation had been performed were analysed. UBM was performed in a standard manner, using a Humphrey UBM 840 system. The clinical relevance of the UBM findings was determined for the whole series and for the following six subgroups of patients arbitrarily established according to the type and location of pathology: hypotony, pseudophakic uveitis, iris and ciliary body pathology excluding hypotony, pars plana pathology, scleritis and Toxocara uveitis. Findings were classified as positive when they confirmed a suspected diagnosis of lesional process or when they gave essential information. Findings were classified as essential when they led to the diagnosis or when they modified therapeutic intervention. RESULTS: During the study period 111 eyes of 77 patients were included. UBM findings contributed essential information that allowed a diagnosis to be reached or that influenced treatment in 43% of cases. It yielded positive findings in 91% of cases, enabling assessment of morphological changes in the iris, ciliary body, and retroiridal and peripheral vitreous induced by intraocular inflammatory or pseudo-inflammatory disorders. Specific UBM signs, present in all patients, were identified in Toxocara uveitis. The groups of patients that benefited most from UBM examination were those with hypotony (83% essential findings) and opaque media (100% essential findings). CONCLUSION: For uveitis patients with an inflammatory process situated in the iris/ciliary body/pars plana/retroiridal vitreous areas, UBM was of great clinical value and improved the management in a significant manner.

The contribution of indocyanine green angiography to the appraisal and management of Vogt-Koyanagi-Harada disease.

OBJECTIVE: The goal of this study was to analyze indocyanine green angiographic (ICGA) findings in Vogt-Koyanagi-Harada (VKH) disease and to determine their value in assessing choroidal involvement as well as their use for diagnostic and follow-up purposes. DESIGN: Retrospective and prospective observational, interventional case series. PARTICIPANTS: Ten patients with VKH disease documented with, for the retrospective cases, at least one concomitant fluorescein and indocyanine green angiogram and, for the prospective cases, follow-up angiograms performed regularly. TESTING: Indocyanine green angiography was performed according to a standard protocol used for inflammatory disorders. Systemic steroids were used for treatment. MAIN OUTCOME MEASURES: Indocyanine green angiographic findings were correlated with funduscopy, fluorescein angiography, inflammatory activity, disease stage, and response to systemic steroids. RESULTS: In newly diagnosed acute disease with exudative retinal detachments, the main features observed in all three patients were: (1) signs indicating choroidal inflammatory vasculopathy, including choriocapillaris perfusion delay in the very early angiographic phase, perivascular leakage of individual vessels in the early phase, diffusely leaking fuzzy vessels in the intermediate phase, and diffuse choroidal hyperfluorescence in the late phase; (2) hypofluorescent dark dots during the intermediate phase of angiography, either becoming isofluorescent in the late phase of the angiogram or remaining hypofluorescent, probably representing partial or full-thickness granuloma; (3) disc hyperfluorescence indicating severe papillitis; and (4) hyperfluorescent pinpoints in the area of exudative retinal detachment. Recurrences in the six patients with chronically evolving disease did not show the hyperfluorescent pinpoints. Otherwise, they showed the same features, albeit less pronounced, together with peripheral atrophic hypofluorescent lesions. In the two patients with "healed" disease for whom high-dose steroids had been initiated at an early stage, only dark hypofluorescent areas in the intermediate and late phases on the fluorescein angiogram were seen, probably representing choroidal scarring. CONCLUSIONS: Consistent ICGA findings in 10 VKH patients allowed the authors to establish a fairly precise pattern of choroidal involvement. Indocyanine green angiography was especially useful to observe the evolution of choroidal inflammatory involvement and to monitor the effect of steroid therapy.