Search

Purpose: To report the management of Vogt-Koyanagi-Harada (VKH) disease based on indocyanine green angiography (ICGA)., Methods: VKH patients with acute episodes of inflammation (inaugural or recurrent) who had received standard ICGA-guided care were studied retrospectively. Standard of care included high dose systemic corticosteroids at presentation and close ICGA follow-up with addition of immunosuppressive agents and/or intensification of ongoing therapy when recurrent choroidal lesions were detected by ICGA. Visual acuity, number of subclinical recurrences, type and duration of therapy, proportion of quiescent patients after therapy, and ICGA findings were recorded., Results: Nine patients including 8 female and one male subject were studied. Five patients had inaugural disease and 4 presented with recurrent acute episodes. Visual acuity increased from 0.86±0.36 to 1.14±0.34 in the right eyes, and from 0.77±0.34 to 1.05±0.33 in the left eyes. The number of ICGA-detected occult choroidal recurrences amounted to 13. Mean duration of treatment was 30.1±34.6 months leading to recurrence-free status after discontinuation of therapy in 6 cases with mean duration of 29.5 months., Conclusion: Continuous monitoring and aggressive therapy guided by ICGA in VKH disease prolongs treatment as compared to textbook guidelines but offers the prospect of reaching inflammation-free status after discontinuation of therapy. Zero tolerance to subclinical choroidal inflammation avoids irremediable evolution towards sunset glow fundus in patients treated early after the initial acute inflammatory attack.

Fuchs' uveitis is very often diagnosed with substantial delay, which is at the origin of deleterious effects such as unnecessary treatment and its consequences. The aim of this study was to analyse the type and frequency of posterior inflammatory and fluorescein angiographic signs in Fuchs' uveitis in conjunction with other clinical signs. Patients seen at the Centre for Ophthalmic Specialised Care (COS) in Lausanne and the Memorial A. de Rothschild, Clinique Générale-Beaulieu in Geneva between 1995 and 2008 with the diagnosis of Fuchs' uveitis and who had undergone a fundus fluorescein angiography (FFA) were analysed. In addition to FFA signs, the data collected included age, gender, initial and final visual acuities, clinical findings at presentation, mean diagnostic delay and ocular complications. Between 1995 and 2008, 105 patients seen in our centres in Lausanne and Geneva were diagnosed with Fuchs' uveitis. Forty of them (38.1%) had undergone at least one FFA. One patient was excluded because of a concomittant diagnosis of multiple sclerosis. In 28 of 39 patients (71.2%) diagnosis was not reached at presentation with a mean diagnosis delay of 3.67 ± 4.86 years (range: 1 month-24 years). The original erroneous diagnosis was intermediate uveitis in 16 patients (57.1%), posterior uveitis in two patients (7.1%), panuveitis in four patients (14.3%) and anterior granulomatous uveitis in six patients (21.4%). Fluorescein angiography demonstrated the presence of disc hyperfluorescence in 43/44 eyes (97.7%), sectorial peripheral retinal vascular leaking in 6/44 eyes (13.6%) and cystoid macular oedema in 4/44 eyes (9.1%), all of which were seen in eyes having undergone cataract surgery. Fuchs' uveitis was bilateral in 5/39 patients (12.8%). The most frequent clinical signs were vitritis in 42/44 eyes (95.5%), stellate keratic precipitates in 41 eyes (93.2%), posterior subcapsular opacities or cataract in 19 eyes (43.2%), and heterochromia in 19 eyes (43.2%). Fuchs' uveitis is a largely underdiagnosed uveitis, probably because the predominant vitreous involvement is ignored by many ophthalmologists. In addition, the nearly constant inflammatory fluorescein angiography findings reported here such as disc hyperfluorescence and, more rarely, peripheral retinal vascular leaking, are not well known and are not usually associated with Fuchs' uveitis but represent an additional factor leading to misdiagnosis. These findings need to be recognised in order to reduce diagnostic delay.

Purpose: To evaluate clinical and angiographic differences in patients with Vogt-Koyanagi-Harada (VKH) disease during the early 4-month treatment phase with high- or medium-dose systemic corticosteroid therapy., Methods: VKH patients treated at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland (n = 4), or the Department of Ophthalmology, Tokyo Medical and Dental University, Tokyo, Japan (n = 5), underwent a pre-treatment indocyanine green angiography (ICGA) and a follow-up ICGA four months after treatment began. Lausanne patients received high-dose, systemic corticosteroid therapy, with or without immunosuppressive therapy. Tokyo patients received medium-dose systemic corticosteroid therapy that included 3 days of intravenous pulse methylprednisolone. ICGA signs including choroidal stromal vessel hyperfluorescence and leakage, hypofluorescent dark dots (HDD), fuzzy vascular pattern of large stromal vessels and disc hyperfluorescence were retrospectively compared., Results: The pre-treatment ICGA demonstrated that each of the nine patients had choroidal inflammatory foci, as indicated by HDD. At 4-month follow-up, clinical and fluorescein findings had improved almost equally in both groups. HDD had resolved in the Lausanne group but persisted in the Tokyo group. Sunset glow fundus occurred in three of the Tokyo patients and none of the Lausanne patients., Conclusions: Submaximal doses of inflammation suppressive therapy are sufficient to suppress clinically apparent disease but not the underlying lesion process. This explains the propensity for sunset glow fundus in seemingly controlled disease.

Purpose: Fuchs' uveitis is often diagnosed with substantial delay at the origin of deleterious consequences such as unnecessary treatment. The aim of the study was to analyse the type and frequency of posterior inflammatory and fluorescein angiographic signs in Fuchs' uveitis in conjunction with the other clinical signs and evaluate their respective importance in the diagnosis of the disease. In particular, diagnostic delay and erroneous diagnoses were investigated., Patients and Methods: Patients seen in our centers between 1995 and 2008 with the diagnosis of Fuchs' uveitis were analysed. The data collected included age, initial and final visual acuities, clinical findings at presentation, mean diagnostic delay, erroneous diagnoses, laser flare photometry values, fundus and fluorescein angiography manifestations and ocular complications., Results: One hundred and five patients were included. The mean age at diagnosis was 34 years. Twelve patients (11.4%) had bilateral involvement. The mean diagnostic delay was 3.04 +/- 4.30 years. The most frequent clinical signs were vitreous infiltration (97.40%), typical Fuchs' keratic precipitates (94.90%), crystalline lens opacities or cataract (47%), heterochromia (42.60%), ocular hypertension or glaucoma (12.80%). The mean laser flare photometry value at presentation was 9.85 +/- 6.28 ph/ms. Thirty-nine patients (37.14%) had undergone fluorescein angiography showing disc hyperfluorescence in 97.7% and peripheral retinal vascular leakage in 13.6%., Conclusions: Fuchs' uveitis is significantly underdiagnosed likely because vitreous involvement was previously described but not commonly recognized as an association with Fuchs' uveitis in the clinician's mind and therefore has often been given a different diagnostic label. Moreover, the very frequent inflammatory signs on fluorescein angiography such as disc hyperfluorescence and more rarely peripheral retinal vascular leakage, which has not been typically associated with Fuchs' uveitis, appear to represent an additional factor leading to misdiagnosis. Such clinical findings need to be publicised in order to reduce misdiagnosis, and diagnostic delay.

Purpose: Firstly, to give a review of characteristic indocyanine green angiographic (ICGA) signs in Vogt-Koyanagi-Harada (VKH) disease and, secondly, to determine the utility of ICG angiography in the assessment and follow-up of choroidal inflammatory activity during initial high-dose inflammation suppressive therapy and during the tapering of therapy., Methods: We have first reviewed characteristic ICGA signs in VKH. This is followed by a study of four patients with an acute initial VKH uveitis episode who received regular initial and follow-up angiographic examinations for at least 9 months. Classical ICGA signs were recorded at onset and followed for at least 9 months and were correlated with treatment levels. The treatment consisted of high-dose oral corticosteroids (0.8-1.5 mg/kg) preceded by pulse intravenous methylprednisolone (500-1000 mg) for 3 days in hyperacute cases and followed by very slow tapering with the addition of an immunosuppressive agent in cases of insufficient response., Results: The major ICGA signs that were both consistently present and easy to record in the four VKH patients having an acute initial uveitis episode with a pre-treatment angiography and an angiographic follow-up for a minimum of 9 months include (1) early choroidal stromal vessel hyperfluorescence and leakage, (2) hypofluorescent dark dots, (3) fuzzy vascular pattern of large stromal vessels and (4) disc hyperfluorescence. All patients were treated with high-dose inflammation suppressive therapy: in two patients, within 14 and 21 days after initial symptoms, respectively, and in the other two patients, within 6 weeks. Hypofluorescent dark dots, the most constant and easily recordable sign, was very prominent in all cases at presentation. A 90% to complete resolution of dark dots was noted in all four patients after 4 months of therapy. The other three major angiographic signs, early choroidal stromal vessel hyperfluorescence and leakage, indistinct fuzzy vessels at the intermediate angiographic phase and disc hyperfluorescence resolved in all cases within 8 weeks or less of high-dose inflammation suppressive therapy. In three of the four patients, dark dots reappeared after a mean of 7.8 +/- 2.8 months after onset of therapy when the patients were under a mean corticosteroid dose of 13.2 +/- 6.3 mg per day without any significant clinical or fluorescein angiographic signs, indicating subclinical recurrence. An increase in the inflammation suppressive therapy again brought about angiographic resolution of choroidal subclinical disease in all cases., Conclusion: Choroidal inflammation shown by ICG angiography can be suppressed completely by initial high-dose inflammation suppressive therapy. However, recurrent subclinical choroidal inflammation is detected at the end of the tapering period in a high proportion of cases. This indicates that, in the absence of an ICGA follow-up, undetected smoldering subclinical disease may persist, thereby explaining the frequently reported evolution towards sunset glow fundus despite an apparently controlled disease. This is a clear indication that VKH disease should be followed by ICG angiography and, in the case of choroidal subclinical reactivation, a reversal of therapy tapering and an extension of therapy duration should be considered.

Background: By allowing one to detect fluorescence beyond the retinal pigment epithelium, indocyanine green angiography (ICGA) has made it possible to analyse the choroidal vessels. Our aim was to characterize choroidal vasculitis in posterior uveitis using ICGA., Methods: Charts of active posterior uveitis patients with a specific diagnosis seen in the different centers participating in the study who had undergone dual fluorescein and ICG angiography were reviewed. The type of inflammatory involvement of the choroidal circulation at entry and the treatment response on follow-up angiograms were analysed., Results: A total of 129 patients were analysed. Choroidal vasculitis could be subdivided into two main patterns: (1) primary inflammatory choriocapillaropathy and (2) stromal inflammatory vasculopathy. The first pattern consisted of hypofluorescent areas up to the late phase of angiography characteristic for choriocapillaris non-perfusion and included entities such as multiple evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multifocal choroiditis (MC), ampiginous choroidopathy and serpiginous choroidopathy. The second pattern consisted of fuzzy indistinct appearance of vessels in the intermediate angiographic phase and diffuse choroidal hyperfluorescence in the late phase indicating inflammatory vasculopathy of larger choroidal vessels. This pattern was found in all cases of active Vogt-Koyanagi-Harada disease, ocular sarcoidosis and tuberculosis and birdshot chorioretinopathy. In Behçet's uveitis of recent onset, choriocapillaris perfusion delay and fuzzy choroidal vessels without diffuse late choroidal hyperfluorescence was found. In posterior scleritis, enlargement of vorticous veins was an additionnal ICGA sign. Stromal inflammatory vasculopathy always responded to anti-inflammatory therapy. A third group of patients with severe retinal or choroidal inflammation presented with associated secondary inflammatory choriocapillaropathy angiographically identical to the primary involvement., Conclusions: ICGA allowed the hitherto impossible characterization of inflammatory involvement of the choroidal vessels, showing either predominant inflammation of the choriocapillaris or predominant inflammation of the stromal choroidal vessels with or without secondary choriocapillaritis. ICGA will be indispensable for the correct evaluation and follow-up of posterior inflammation with suspected choroidal involvement.

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.

Aim: The Vogt-Koyanagi-Harada (VKH) syndrome is characterized by a bilateral granulomatous uveitis with exudative retinal detachments associated with systemic manifestations such as meningeal signs, cutaneous signs (poliosis, alopecia and vitiligo) and dysacousis. VKH is relatively unfrequent in Europe and Switzerland. Therefore diagnosis is often reached with some delay. Our aim here was to analyze the 3 patients for whom the diagnosis was reached less than 15 days after the first signs and compare their evolution to seven patients for whom diagnosis was known one month or more after the first signs., Patients and Methods: Retrospective and partially prospective study of patients seen at uveitis clinic in Lausanne from 1990 to 1999 for whom an ICG angiographic work-up had been performed in addition to the usual clinical and fluoresceinic work-up. The frequency of VKH in our collective was calculated; symptoms and signs, paraclinical investigations, laboratory work-up, delay from first signs to diagnosis, the management and the evolution were the criteria analyzed. In particular the patients with early diagnosis and early treatment were analyzed and compared to the rest of the collective. Diagnosis was based on the criteria of the American Uveitis Society. Between 1990 and 1999, 14 patients with the diagnosis of VKH were seen (1.2% of our collective of uveitis patients). The 10 patients having had a work-up including ICG angiography in addition to the classical work-up were included in this study., Results: The diagnosis was reached in less than 2 weeks in 3 patients. In all 3 patients inflammation was controlled after treatment. Two patients with a follow-up without recurrence of respectively 36 and 54 months were considered as healed. The last case had no recurrence after nine months but still was under therapy. Whereas clinical examination and fluorescein angiography failed to show any sequels in the 2 "healed" patients, ICG angiography showed numerous zones of hypofluorescence indicating choroidal scarring. For the 7 other cases, the diagnosis was reached one month or more after the first symptoms or signs and they all evolved in the chronic recurrent fashion. ICG angiography contributed to the rapid diagnosis in 2/3 patients with early diagnosis and was an essential parameter for the choroidal follow-up in 9/10 patients., Conclusion: This study shows that it is essential to rapidly reach the diagnosis of VKH and treat the patients vigorously without delay. By showing choroidal lesions not seen by the clinical examination or fluorescein angiography. ICG angiography is essential for a correct work-up and follow-up of choroidal lesions in VKH. In our two "healed" patients it was the only mean to show choroidal sequellae.

Disseminated Mycobacterium-avium complex (MAC) infection develops in most patients with AIDS. We report three cases of anterior uveitis with vitritis in AIDS patients treated by the combination of rifabutin, clarithromycin and ethambutol for MAC bacteremia. Uveitis secondary to the introduction of rifabutin treatment is suggested.