Vogt–Koyanagi–Harada syndrome in a Greenlandic Inuit

Purpose: To report a case of Vogt–Koyanagi–Harada syndrome (VKH) in an Inuit. Methods: We carried out a medical evaluation and human leucocyte antigen (HLA) genotyping. Results: A 36‐year‐old male Inuit developed severely decreased vision, intense headache and vertigo over a 3‐week period. Ocular examination revealed panuveitis with bilateral serous retinal detachment and optic nerve head oedema. There was no history of ocular trauma or evidence suggestive of other disease entities. The patient responded well to high‐dose systemic prednisolone. Vitiligo presented late in the course. Conclusions: This case report describes the first published case of VKH in a patient of Inuit ancestry. The patient was homozygous for HLA‐DR4, a genotype previously associated with VKH.     

Effect of the duration of immunomodulatory therapy on the clinical features of recurrent episodes in Vogt–Koyanagi–Harada disease

Purpose: To evaluate the duration of immunomodulatory therapy (corticosteroids, immunosuppressive drugs) with regard to the rate of relapses and clinical features (exudative retinal detachment or anterior uveitis) in inflammatory episodes of Vogt–Koyanagi–Harada disease. Methods: Data of all 42 patients diagnosed with acute uveitis associated with VKH disease during the period of January 2005 to December 2008 at the Pitié‐Salpêtrière Hospital or at the Lariboisière Hospital in Paris, France were extracted by chart review. Results: There were 31 patients (73.8%) with episodes of recurrence and were included in the study. At the first recurrence, 81% (13 patients) of exudative retinal detachments (ERD) were associated with an initial immunomodulatory treatment conducted ≤6 months (3.76 months ± 2.67). Conversely, an initial treatment duration of >6 months was associated with anterior uveitis signs for 66% of patients (eight patients) with anterior first recurrence (p = 0.0061). On second episode of recurrence, 75% of patients (three patients) who developed exudative retinal detachment had been managed by immunomodulatory therapy for ≤6 months with the total duration of immunomodulatory treatment ≤6 months during previous inflammatory episodes. Conversely, all 16 patients who presented anterior uveitis with additional manifestations (optic disc oedema, macular oedema, vitritis and/or ‘Sunset glow’ fundus) have been treated for more than 6 months or treated during the initial occurrence lasting more than 9 months (p = 0.0035). Conclusions: The duration of systemic corticosteroids (and/or immunosuppressive drug therapy) for ≤6 months at first and second recurrence was associated with features of further exudative retinal detachment instead of anterior uveitis in VKH disease.     

Prognostic factors for clinical outcomes in patients with Vogt–Koyanagi–Harada disease treated with high‐dose corticosteroids

Purpose: To determine prognostic factors in patients with Vogt–Koyanagi–Harada (VKH) disease who were treated with high‐dose corticosteroids. Methods: Retrospective analysis of 87 patients (174 eyes). Results: At presentation, there were 53 patients with initial‐onset acute VKH disease and 34 patients with chronic recurrent VKH disease. Chronic recurrent presentation was significantly associated with more severe anterior segment inflammation at presentation as indicated by presence of mutton‐fat keratic precipitates, anterior chamber reaction ≥2+, iris nodules and posterior synechiae (p < 0.001 for all comparisons), less exudative retinal detachment at presentation (p < 0.001), more complications during the follow‐up period (p < 0.001) and a worse visual outcome (p < 0.001). The use of immunomodulatory therapy (cyclosporine and mycophenolate mofetil) as first‐line therapy significantly reduced the development of complications in the whole study group (p = 0.006) and in initial‐onset acute group (p = 0.024) and improved visual outcome in the whole study group (p = 0.004) and in chronic recurrent group (p = 0.024). In the whole study group, final visual acuity of 20/20 was significantly associated with good initial visual acuity of >20/200 [odds ratio = 4.25; 95% Confidence interval (CI) = 1.53–11.89] and age older than 16 years was significantly associated with the development of complications (odds ratio = 3.15; 95% CI = 1.04–9.48). Conclusions: Chronic recurrent VKH disease is significantly associated with more severe anterior segment inflammation and less exudative retinal detachment at presentation, more ocular complications and a worse visual outcome than initial‐onset acute VKH disease. Use of immunomodulatory therapy significantly improved the clinical outcomes.     

The outcomes of mycophenolate mofetil therapy combined with systemic corticosteroids in acute uveitis associated with Vogt–Koyanagi–Harada disease

Purpose: To study the effectiveness of mycophenolate mofetil (MMF) as first‐line therapy combined with systemic corticosteroids in acute uveitis associated with Vogt–Koyanagi–Harada (VKH) disease. The outcomes in this group were compared with those of another group of patients with VKH disease who were treated with corticosteroid monotherapy or with delayed addition of immunomodulatory therapy. Methods: This prospective study included 19 patients (38 eyes) diagnosed with acute uveitis associated with VKH disease. Results: The mean follow‐up period was 27.0 ± 11.1 months (range 16–54 months). Corticosteroid‐sparing effect was achieved in all patients. The mean interval between starting treatment and tapering prednisone to 10 mg or less daily was 5.1 ± 1.2 months (range 3–7 months). Ten (53%) patients discontinued treatment without relapse of inflammation. The mean time observed of treatment was 17.3 ± 11.9 months (range 3–41.5 months). Visual acuity of 20/20 was achieved by 38% of the eyes in the corticosteroid group and by 74% in the corticosteroid + MMF group (p < 0.001). Recurrent inflammation of ≥3 times was reduced significantly (p = 0.0383) in the corticosteroid + MMF group (3%) as compared to corticosteroid group (18%). Development of all complications was significantly higher in the corticosteroid group (43%) compared with the corticosteroid + MMF group (8%) (p < 0.001). None of the eyes in the corticosteroid + MMF group developed ‘sunset glow fundus’. Conclusions: Addition of MMF as first‐line therapy to corticosteroids in patients with acute uveitis associated with VKH disease leads to significant reduction in recurrences of uveitis and development of late complications and significantly improves visual outcome.     

Elevated choroidal blood flow velocity during systemic corticosteroid therapy in Vogt–Koyanagi–Harada disease

Purpose: Laser speckle flowgraphy (LSFG) can be used to non‐invasively visualize the haemodynamics of choroidal circulation and the vascular pattern. The purpose of this study was to examine the ability of LSFG to quantitatively evaluate blood flow velocity at the macula in patients with Vogt–Koyanagi–Harada (VKH) disease before and after systemic corticosteroid therapy. Methods: Prednisolone (200 mg/day) was systemically administered in 10 VKH disease patients with serous retinal detachment at the macular area. The drug was gradually tapered to zero over a 6‐month period. Laser speckle flowgraphy measurements were taken in the 20 eyes of these patients at their initial visit and at 1, 4 and 12 weeks after the onset of therapy. Square blur rate (SBR), a quantitative index of relative blood flow velocity, was calculated using LSFG. Results: Serous retinal detachment resolved within 4 weeks after treatment and visual acuities improved to > 1.0 in almost all cases. There were significant increases in average SBR at the macula at 4 weeks after treatment compared with at 1 week after treatment, and also at 12 weeks after treatment compared with at 4 weeks after treatment. Conclusions: These results suggest that systemic corticosteroid therapy improves inflammation‐related impairment in choroidal blood flow velocity at the macula. Laser speckle flowgraphy can evaluate the effect of systemic corticosteroid therapy by enabling comparisons between measurements of blood flow velocity, which is considered to reflect inflammation activity in the choroid.     

原田病药物治疗后吲哚青绿眼底血管造影观察

目的 探讨利用吲哚青绿血管造影检查作为原田(Harada)病临床观察指标的意义.方法 对经药物治疗后19例(38只眼)原田病患者同时行荧光素眼底血管造影(fundus fluorescein angiography,FFA)及吲哚青绿血管造影(indocyanine green angiography,ICGA)检查资料进行分析.结果 FFA检查发现有4例(8只眼)视盘轻度染色,视网膜未发现异常改变;ICGA早期及中期无异常表现,但后期8只眼(包括FFA中视盘染色的4只眼)在中周及后极部出现了点状或斑片状弱荧光;3例6只眼FFA及眼底正常,在ICGA后期后极部出现了斑点状弱荧光;24只眼脉络膜大、中血管影像减少,其中12只眼dalen-fuchs结节着色.结论 ICGA可较好提供原田病的脉络膜循环损害的信息,并在评价疗效上有意义.

Abstract：

Objective To investigate the clinical value of indocyanine green angiography(ICGA)in patients with Harada disease.Methods Fundus fluorescein angiography(FFA)and indocyanine green angiography(ICGA)were used for comparative analyses in 26 cases(52 eyes)of Harada disease after treatment.Results Mild dyeing in disk was found only in 4 cases(8 eyes),and the others were not found any abnormal in FFA.There were no any abnormal found in early period and middle period of ICGA,but of 8 eyes(include 4 eyes of dyeing in disk in FFA)multifocal lower fluorescence were found in the mid-periphery and posterior pole of the fundus in the late phase.Of 3 cases(6 eyes)were normal in FFA,however multifocal lower fluorescence were found in the posterior pole of the fundus in the late phase of ICGA.Of 24 eyes were found decreased fluorescence in large and middle choroidal vessel,and 12 eyes of Dalen-Fuchs were colored.Conclusions ICGA may assist in providing valuable information on choroidal circulation of Harada disease and be useful in evaluating the curative effects.     

Vitreous surgery for macular hole in patients with Vogt‐Koyanagi‐Harada disease

We describe two patients with Vogt‐Koyanagi‐Harada (VKH) disease, both in the convalescent stage, who presented with unilateral macular holes together with clinically significant epi‐retinal membranes. Vitreo‐retinal surgery was performed on the affected eyes and the surgical technique involved a standard three‐port vitrectomy, peeling of the epi‐retinal and internal limiting membrane (ILM). In both cases the retinae were tamponaded with air resulting in anatomical closure of the macular holes. The histology of the excised membrane was available in one case and this revealed multiple layers of presumed retinal pigment epithelial cells with cytoplasmic processes and intercellular junctions forming a basal lamina attached to the smooth surface of the ILM. Our findings demonstrate that macular holes can develop in patients with VKH but that the hole can be successfully closed with vitreo‐retinal surgery. The convalescent stage tends to occur several weeks after the acute stage when the uveitic process has subsided and is characterized by choroidal depigmentation, producing a sunset glow appearance to the ocular fundus. Patients may also demonstrate varying degrees of cutaneous hypopigmentation, poliosis and/or alopecia. Macular holes have also been reported previously in patients during the convalescent stage of VKH and this communication describes the outcome of two patients who underwent vitreo‐retinal surgery for this problem.     

Indocyanine green angiographic findings in central serous chorioretinopathy

There has been great controversy about the pathogenesis of the focal changes in the retinal pigment epithelium (RPE) causing detachment of the neurosensory retina in central serous chorioretinopathy (CSC). This study was performed to evaluate changes in choroidal perfusion in 15 patients with CSC. Fluorescein and indocyanine green (ICG) angiography was performed in patients with acute or chronic recurrent CSC. In all patients delayed arterial filling followed by capillary and/or venous congestion, in some cases adjacent to ischemic areas, was found in the choroid. Leakage from the RPE in fluorescein angiography was only observed in those areas with choroidal capillary and/or venous congestion. The preliminary results suggest that venous congestion possibly in association with ischemia causes hyperpermeability of choroidal vessels already described in the literature.     

Indocyanine green angiographic findings in idiopathic choroidal neovascularization

Purpose. Evaluation of choroidal alterations associated with idiopathic choroidal neovascularization (ICNV) and the possible relation between this affection and Multifocal Choroidopathies (MC). Methods. The authors analysed, using high definition videoangiography, the choroidal findings in 21 consecutive patients affected by ICNV (7 males and 14 females; 19–46 years; mean age: 31.8 years); with a follow-up at 5–30 months (mean 13 months). Moreover, a retrospective study of 20 cases of ICNV (11 males and 9 females; age: 17–39 years; mean age: 29.3 years), with a follow-up at 6–11 years (mean 8.9), was performed. Results. In 7 eyes, the indocyanine green angiography (ICGA) showed choroidal hypofluorescent spots similar to those observed in MC (in 3 cases even in the fellow unaffected eye), in 2 of them the regression of the spots was observed after steroid therapy. In 2 eyes, the ICGA revealed hyperfluorescent spots; in one of them the complete regression of the spots after oral cyprofloxacine was observed. In 6 patients (10 eyes), choroidal permeability alterations could be visualized (in 4 cases even in the unaffected eye). Conclusions. The indocyanine green angiographic findings (hypo and hyperfluorescent spots, choroidal permeability alterations) could support the theory of Gass which considers that ICNV is not idiopathic but secondary to a widespread choroidal inflammatory disease. The similarity of the ICGA alterations in ICNV and MC, the observation that cases of ICNV would become MC in the follow-up, could allow the hypothesis of a close connection between these two affections.     

Indocyanine green angiographic findings of Dalen-Fuchs nodules in Vogt-Koyanagi-Harada disease

Purpose The purpose of this study was to analyze indocyanine green (ICG) angiographic findings of Dalen-Fuchs nodules in Vogt-Koyanagi-Harada (VKH) disease. Methods ICG angiograms of 15 patients (30 eyes) with Dalen-Fuchs nodules in VKH disease of between 2 months and 5 years after the initial diagnosis were retrospectively studied. Findings of ICG angiography were compared with features of fundus fluorescein angiography (FFA). Results Dalen-Fuchs nodules were easily found in the inferior (30 eyes, 100%) and temporal periphery (22 eyes, 73%) and showed two kinds of fluorescence in ICG angiography. In ten patients (20 eyes), the nodules showed small round hypofluorescent dark dots in the whole process of angiography, and the dark dots were larger in size than the nodules in FFA. Disease course in these patients was relatively long—between 1 year and 5 years. On clinical examination, the nodules were atrophic, and hyperpigmentation was found around them. In another five patients (ten eyes), parts of the nodules showed small hyperfluorescent dots in the early phase, but they were faint in the intermediate phase and became large, hypofluorescent dark dots in the late phase. Disease course in these patients was between 2 and 8 months. The nodules were bright yellow, fresh, and much larger than those in the first kind of ICG fluorescence. Conclusions Dalen-Fuchs nodules in VKH are mostly present in the inferior and temporal periphery. The two kinds of fluorescence of Dalen-Fuchs in ICG angiography may reflect obliteration of choriocapillaris under the nodules and different quantities of lipofuscin in the nodules at different time points of the disease.     

Indocyanine green angiographic findings in fellow eyes of patients with unilateral occult neovascular age-related macular degeneration

Purpose: To determine the role of indocyanine green angiogaphy (ICGA) in the prediction of the development of choroidal neovascularization (CNV). Methods: We reviewed the ICG angiograms of 124 patients with newly diagnosed unilateral occult CNV secondary to age-related macular degeneration (AMD) in whom the fellow eye had only drusen by biomicroscopy and fluorescein angiography. Follow-up data of at least 12 months were obtained in all eyes. Results: 113 fellow eyes had a normal ICG study, while 11 fellow eyes revealed plaque-like late hyperfluorescence. During an average follow-up time of 18.2 months exudative AMD developed in 7 of 11 eyes with late hyperfluorescence on ICGA and in 6 of 113 with a normal ICGA study. Conclusion: ICGA may help to predict which fellow eyes are at higher risk of developing future exudative changes. This revised version was published online in September 2006 with corrections to the Cover Date.     

Indocyanine green angiographic findings of obscure choroidal abnormalities in neurofibromatosis

We report two cases of choroidal neurofibromatosis, detected with the aid of indocyanine green angiography (ICGA) in patients with neurofibromatosis (NF)-1, otherwise having obscure findings based on ophthalmoscopy and fluoresceine angiography (FA). In case 1, the ophthalmoscopic exam showed diffuse bright or yellowish patched areas with irregular and blunt borders at the posterior pole. The FA showed multiple hyperfluorescent areas at the posterior pole in the early phase, which then showed more hyperfluorescence without leakage or extent in the late phase. The ICGA showed diffuse hypofluorescent areas in both the early and late phases, and the deep choroidal vessels were also visible. In case 2, the fundus showed no abnormal findings, and the FA showed weakly hypofluorescent areas with indefinite borders in both eyes. With the ICGA, these areas were more hypofluorescent and had clear borders. Choroidal involvement in NF-1 seems to occur more than expected. In selected cases, ICGA is a useful tool to be utilized when an ocular examination is conducted in a patient that has no definite findings based on the ophthalmoscope, B-scan, or FA tests.     

Indocyanine green angiography in posterior uveitis

Literature review for indocyanine green angiography and evaluate the role of indocyanine green angiogram (ICGA) in patients with posterior uveitis seen at a tertiary referral eye care centre. Detailed review of the literature on ICGA was performed. Retrospective review of medical records of patients with posterior uveitis and dual fundus and ICGA was done after institutional board approval. Eighteen patients (26 eyes) had serpiginous choroiditis out of which 12 patients had active choroiditis and six patients had healed choroiditis, six patients (12 eyes) had ampiginous choroiditis, six patients (12 eyes) had acute multifocal posterior placoid pigment epitheliopathy, eight patients (10 eyes) had multifocal choroiditis, four patients (eight eyes) had presumed ocular histoplasmosis syndrome, four patients (eight eyes) had presumed tuberculous choroiditis, two patients (four eyes) had multiple evanescent white dot syndrome and two patients (four eyes) had Vogt Koyanagi Harada (VKH) syndrome. The most characteristic feature noted on ICGA was the presence of different patterns of hypofluorescent dark spots, which were present at different stages of the angiogram. ICGA provides the clinician with a powerful adjunctive tool in choroidal inflammatory disorders. It is not meant to replace already proven modalities such as the fluorescein angiography, but it can provide additional information that is useful in establishing a more definitive diagnosis in inflammatory chorioretinal diseases associated with multiple spots. It still needs to be determined if ICGA can prove to be a follow up parameter to evaluate disease progression.     

Indocyanine green angiographic and optical coherence tomographic findings support classification of polypoidal choroidal vasculopathy into two types

Purpose: We assessed the characteristic indocyanine green angiographic (ICGA) and spectral domain optical coherence tomographic (SD‐OCT) findings of two types of polypoidal choroidal vasculopathy (PCV), distinguishable by different filling patterns on ICGA. Methods: Thirty‐one eyes with PCV were classified into types 1 and 2 based on ICGA findings of either the presence or absence of both a feeder and a draining vessel. Characteristic ICGA findings were evaluated for each type of PCV. Spectral domain optical coherence tomographic images of the 31 eyes were also used to compare the two types of PCV. Results: Both a feeder and a draining vessel were observed in 13 eyes (type 1). Eighteen eyes had neither feeder nor draining vessels (type 2). In PCV type 1, a break in the highly reflective line thought to be Bruch’s membrane was detected, corresponding to the feeder vessel in‐growth site on SD‐OCT. This line was straight. In PCV type 2, the highly reflective line exhibited irregular thickness and had highly reflective substances adhering to its lower portion. It curved downward and became increasingly obscure, ultimately disappearing at a point corresponding to the site at which network vessel filling began. The mean subfoveal choroidal thicknesses in eyes with PCV type 1 and PCV type 2 were 199 ± 65 and 288 ± 98 μm, respectively. Conclusions: Our observations support the existence of two distinct types of PCV. The first type represents choroidal neovascularization, whilst the second type involves choroidal vasculature abnormalities.