To report the management of Vogt-Koyanagi-Harada (VKH) disease based on indocyanine green angiography (ICGA).
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.
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.
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.
Vogt-Koyanagi-Harada Disease; Indocyanine Green Angiography; Therapy
We report a female patient diagnosed as Vogt-Koyanagi-Harada (VKH) and polycystic ovary syndrome (PCOS). She has diagnosed as VKH with diminished vision, bilateral serous retinal detachment, the signs of fundus fluorescein angiography and the findings of optical coherence tomography. The patient was referred to the gynecology clinic for her complaints as weight gain, hirsutismus and amenorrhea. She has also been diagnosed with PCOS. With oral steroid treatment, visual acuity has improved and the detachments have resolved within a month. VKH disease may be associated with polycystic ovary syndrome. The two conditions may have a common autoimmune pathogenesis.
Autoimmune pathogenesis; Polycystic ovary syndrome; Vogt-Koyanagi-Harada
BACKGROUND/AIM—The significance of indocyanine green (ICG) angiography (ICGA) in Harada's disease still awaits clarification in many respects. This study investigates the details of choroidal lesions observed in Harada's disease by the subtraction method.
METHODS—Eight patients with Harada's disease were followed with ICGA. ICG angiograms were obtained with a Topcon high resolution digital fundus camera and processed with a Topcon IMAGEnet computer system. Image subtraction was conducted for analysing serial angiograms taken at about 2 second intervals during the dye transit phase and those taken in the early and middle phases of angiography.
RESULTS—Standard ICG images of acute stage disease showed delayed choroidal filling in the early phase. Mid phase angiograms showed areas with bright fluorescence of variable intensity, indicating intrachoroidal ICG leakage. With image subtraction of angiograms with an interval of seconds the choroidal vessels could be imaged sequentially, with the choroidal arteries visualised first, followed by the definition of the choriocapillaris and then the choroidal veins. The choroidal veins with delayed filling were visualised as positive images in serial subtraction angiograms. Subtraction with an interval of minutes showed uneven background fluorescence and bright fluorescence corresponding to the areas of intrachoroidal ICG leakage. After the disease subsided with steroid therapy, angiography revealed an improvement in delayed choroidal filling. Image subtraction by the second allowed a clear visualisation of improved choroidal venous filling, while subtraction by the minute showed homogeneous background fluorescence, eliminating brighter areas.
CONCLUSION—Subtraction ICGA demonstrated that delayed filling of the choroidal veins of varying severity occurs in association with hyperpermeability of the choroidal vessels in the course of Harada's disease.
AIMS--Analysis of the choroidal findings in patients affected by serpiginous choroidopathy (SC). METHODS--Thirteen patients (23 eyes; 11 males and two females; age range 50-68 years; mean age 59.1 years) affected by SC were examined with fluorescein angiography (FA) and indocyanine green angiography (ICGA). The follow up period was 7-33 months. RESULTS--Using ICGA the disease could be divided into the following stages: (1) subclinical or choroidal stage (hypofluorescent lesions without FA evidence); (2) active stage (with ICGA and FA evidence); (3) subhealing stage (slight late hyperfluorescent lesions with ICGA, with no evidence on FA); (4) inactive or healed stage (hypofluorescent areas with ICGA and hyperfluorescent areas with FA). CONCLUSIONS--Although FA showed a clear distinction between active and healed stages, ICGA allowed a greater subdivision of the disease. In particular, ICGA allowed: (1) better staging of SC, revealing choroidal alterations when there was no ophthalmoscopic or FA evidence; (2) better identification of the active lesions which appear to be larger at the choroidal level in comparison with the corresponding retinal lesions; and (3) revealed a persistence of choroidal activity even when the signs of retinal activity had disappeared. Thus, ICGA should be a particularly useful clinical and therapeutic monitoring tool of SC.
Central serous chorioretinopathy (CSC) is a condition that originates from alterations of the choroidal circulation. The aim of this paper was to evaluate the use of indocyanine green angiography (ICGA) in patients with chronic CSC.
The analysis included 17 patients (34 eyes) with chronic CSC in at least 1 eye. The eye examination included: distance and near visual acuity, biomicroscopy, applanation tonometry, fundus examination, colored and red-free fundus photography, evaluation of autofluorescence, optical coherence tomography, and fluorescein and indocyanine green angiography.
In 34 eyes (100%) involved in the ICGA study the results revealed zones of transient increased choroidal vessels permeability. In 18 eyes (52.9%) choroidal changes were accompanied by a focal serous pigment epithelial detachment. In 4 eyes (11.8%) of 3 patients’ the ICGA examination confirmed the presence of occult choroidal neovascularization (CNV). In the patient with bilateral diffuse retinal pigment epitheliopathy, CNV was present in 1 eye, in the patient with unilateral chronic CSC it was also present in 1 eye, and in the third patient with bilateral chronic CSC it was detected in both eyes.
ICGA is a very useful examination that enables ophthalmologists to visualize choroidal changes due to chronic CSC, as well as to diagnose occult CNV in chronic CSC.
central serous chorioretinopathy; occult choroidal neovascularization; indocyanine green angiography; optical coherence tomography; pigment epithelium detachment
AIM—To observe pathophysiological changes in patients with Vogt-Koyanagi-Harada (VKH) syndrome using the scanning laser ophthalmoscope (SLO) to perform videofunduscopy, and indocyanine green (ICG) and fluorescein videoangiography.
METHODS—18 patients diagnosed with VKH syndrome were studied. 15 patients were examined in both acute and convalescent phases, and three patients were examined in the convalescent phase only. Retrospective review of charts was performed to obtain data on visual acuities, clinical findings, and results of cerebrospinal fluid cytology and histocompatibility leucocyte antigen D locus testing.
RESULTS—Videofunduscopy revealed abnormalities in the central macula at the level of the retinal pigment epithelium or choroid in 40% of patients in the acute phase and 83% of patients in the convalescent phase. ICG videoangiography showed irregular hypofluorescence (80%), slow or patchy filling (67%), indistinct or fewer choroidal vessels (73%), filling defects (73%), and focal leakage (33%) in patients in the acute phase. These changes improved with systemic corticosteroid treatment, although at least one ICG videoangiographic abnormality persisted into the convalescent phase in 83% of patients in the absence of clinical inflammation. In contrast, fluorescein videoangiography revealed fewer and decreased severity of abnormalities, with greater resolution in the convalescent phase when compared with ICG videoangiography.
CONCLUSIONS—SLO videofunduscopy and videoangiography revealed numerous abnormalities in patients with VKH syndrome, many of which persisted well after clinical recovery, suggesting the development of permanent morphological changes in the fundus. ICG videoangiography was more sensitive than fluorescein videoangiography in delineating abnormalities in layers deep to the sensory retina.
Keywords: fluorescein; fundus angiography; indocyanine green; Vogt-Koyanagi-Harada syndrome
To describe the pathoanatomy of diabetic choroidopathy (DC) in pre-diagnosed diabetic retinopathy (DR) cases and to provide angiographic and optical evidence for DC using indocyanine green angiography (ICGA) and enhanced depth imaging spectral-domain optical coherence tomography (EDI SD-OCT).
A retrospective analysis of 80 eyes from 40 DR patients was conducted. In Group One, choroidal vascular abnormalities were evaluated by comparing angiographic findings from simultaneous ICGA with those from fundus fluorescein angiography (FFA). In Group Two, EDI SD-OCT was used to evaluate the subfoveal choroidal thickness (SFCT) and define the choroid boundary in order to acquire the bilateral and symmetric choroidal area (CA). Data were then analyzed by Image Pro Plus 6.0.
In Group One, choroidal abnormalities that were evident using ICGA but not FFA included early hypofluorescent spots in 47 eyes (75.81%), late hyperfluorescent spots in 37 eyes (59.68%), and late choroidal non-perfusion regions in 32 eyes (51.61%). In particular, a significant difference between proliferative DR (PDR) in 17 of 23 eyes (73.91%) and non-PDR in 16 of 39 eyes (41.03%) was observed in late choroidal non-perfusion regions. Eighteen of 31 eyes (58.06%) also exhibited “inverted inflow phenomena.” In Group Two, both the SFCT and CA of eyes with diabetic macular edema and serous macular detachment were significantly greater than those in the other eyes. The CA in panretinal photocoagulation (PRP) treated cases was also greater than that in non-PRP treated cases.
Early hypofluorescent spots, late choroidal non-perfusion regions, inverted inflow phenomena, higher SFCT, and larger CA are qualitative and quantitative indexes for DC. Moreover, the late choroidal non-perfusion region is a risk factor for DC with DR. Our study suggests that the supplemental use of ICGA and EDI SD-OCT with FFA is a better choice for DR patients.
We describe a case of bilateral exudative retinal detachment associated with prodromal symptoms simulating the presentation of acute Vogt-Koyanagi-Harada disease that was eventually diagnosed as acute lymphoblastic leukemia. A 42-year-old man presented with sudden visual loss in both eyes for two weeks. He complained of intermittent headache, neck stiffness and tinnitus for a month. His best-corrected visual acuities were 20/200 in both eyes. Fluorescein angiography, optical coherence topography and indocyanine green angiography featured bilateral serous retinal detachments. A clinical diagnosis of incomplete type Vogt-Koyanagi-Harada disease was considered. However, complete blood cell count showed a marked increase in the number of white blood cells and bone marrow examination revealed precursor B cell lymphoblastic leukemia. The patient started on induction chemotherapy. A week later, his best-corrected visual acuities were 20/25 and the serous retinal detachments were nearly absorbed in both eyes. Bilateral exudative retinal detachment associated with neurologic and auditory abnormalities may be a presenting sign of acute lymphoblastic leukemia. Clinicians should be aware of the possibility of leukemia in such patients.
Exudative retinal detachment; Leukemia; Vogt-Koyanagi-Harada disease
The purpose of this study is to assess indocyanine green angiographic findings in patients with nonfamilial amyloidosis.
The method used was a prospective study including seven patients (14 eyes) with nonfamilial amyloidosis. All patients underwent detailed ophthalmic clinical examination, fundus photography, and indocyanine green angiography (ICGA). Fluorescein angiography (FA) was performed in four patients.
Of the seven patients, four (57.1 %) were male. Mean age was 49.5 years. Six patients had renal amyloidosis and one patient had systemic amyloidosis. Mean best-corrected visual acuity was 20/25. Fundus and FA findings included cotton-wool spots (28.5 %), retinal hemorrhages (14.3 %), retinal pigment epithelial changes (21.4 %), serous retinal detachment (7.1 %), optic disk edema or staining (7.1 %), area of peripheral retinal capillary non-perfusion (7.1 %), disseminated peripheral punctiform hyperfluorescence (21.4 %), and subretinal pooling (7.1 %). Fundus examination results were unremarkable in eight eyes (57.1 %). ICGA showed abnormal findings in all eyes. These included diffuse or focal/multifocal choroidal vascular staining appearing at the late phase and prevailing in peripheral fundus (100 %), hyperfluorescent fleecy lesions appearing at the late phase and also prevailing in peripheral fundus (28.5 %), hypofluoresent areas of variable sizes (85.7 %), and pinpoints (71.4 %).
Our results show that a subclinical, fairly typical choroidal involvement, detectable only by ICGA, is common in patients with nonfamilial amyloidosis. ICGA may be useful in better understanding the pathogenesis of amyloidosis choroidopathy and in establishing a diagnosis of amyloidosis in atypical or incomplete clinical presentations.
Choroidopathy; Indocyanine green angiography; Nonfamilial amyloidosis
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.
Choroid; Choroidal neurofibroma; Indocyanine green angiography; Neurofibromatoses
To present a patient with overlapping choriocapillaritis syndromes who first presented as a typical case of multiple evanescent white dot syndrome (MEWDS) and later with characteristic findings compatible with multifocal choroiditis (MFC).
A 40-year-old myopic woman presented with a paracentral scotoma OS. Fundus examination revealed pale discolored areas around the optic disc corresponding to faintly hyperfluorescent areas on fluorescein angiography (FA). On indocyanine green angiography (ICGA) there was extensive peripapillary hypofluorescence and confluent hypofluorescent dots superiorly. According to the clinical picture, a diagnosis of MEWDS was made. In 4 weeks, the visual field reverted to normal together with almost complete regression of hypofluorescence on ICGA. However, 4 months later fundus examination revealed some scars, a finding not typical for MEWDS. Besides, she developed another scotoma 12 months later accompanied by photopsia and the fundus illustrated more numerous scars than one year earlier. ICGA showed hypofluorescent areas corresponding to the scotoma delineated by visual field testing. The pattern of this recurrence clearly corresponded to MFC.
This case illustrates an overlap between two entities, MEWDS and MFC in two sequential episodes. FA and fundus autofluorescence accounted for the lesions and optical coherence tomography showed damage to the photoreceptor outer segments, but only ICGA correlated well with functional evolution.
Choriocapillaritis; Multiple Evanescent White Dot Syndrome; Multifocal Choroiditis Indocyanine Green Angiography; Optical Coherence Tomography; Fundus Autofluorescence
To present the clinical, indocyanine green angiography (ICGA) features and results of treatment for polypoidal choroidal vasculopathy (PCV) in Indian patients by a retrospective chart review.
Materials and Methods:
Forty five patients with PCV underwent complete ocular examination, fluorescein angiography (FFA) and ICGA. Treatment was advised for patients with macular involvement and progressive loss of visual acuity. Demographic data, clinical features and results of treatment were analyzed.
Mean age at presentation was 61.06 years. Mean follow up was 18 months. The disease was more prevalent in males. Forty three patients had unilateral disease. The most common location of polyps in ICGA was subfoveal (42.5%). Exudative form was seen in 34 of the 47 eyes and the remaining 13 eyes had a hemorrhagic presentation. Thirty four eyes underwent treatment which included thermal laser (n = 11), photodynamic therapy (PDT) (n = 11) and transpupillary thermo therapy (TTT) (n = 12). Statistical analysis was done using the Chi-square test. Subgroup analysis of visual outcome following various modalities of treatment showed that the results of PDT (P < 0.001) and thermal laser (P < 0.001) were statistically significant.
PCV is an important differential diagnosis in patients presenting with serosanginous maculopathy and submacular hemorrhage. The disease was more prevalent in males and was unilateral in the Indian population. Timely intervention in cases with symptomatic polyps could achieve stabilization of visual acuity. Thermal laser and PDT were safe and effective.
Greatest linear diameter; indocyanine green angiography; photodynamic therapy; polypoidal choroidal vasculopathy; serosanguinous maculopathy; submacular hemorrhage; thermal laser
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.
Ampiginous choroiditis; fluorescein angiography/fluorescein angiogram; indocyanine green angiogram; multifocal choroiditis; posterior uveitis; serpiginous choroiditis
Vogt-Koyanagi-Harada (VKH) disease is characterized by bilateral granulomatous uveitis with neurologic, auditory, and dermatologic manifestations. However, acute myelitis complicating VKH disease has rarely been reported.
A 50-year-old Chinese Han woman presented with difficulty walking, numbness on the left side of the body, and difficulty with urination. The patient was diagnosed with incomplete VKH disease and received corticosteroid treatment prior to the neurological presentation. Acute myelitis was diagnosed based on both clinical and spinal-cord MRI findings.
Clinicians should consider acute myelitis as a rare possible neurological manifestation in VKH disease patients, and early systemic administration of corticosteroids will suppress the acute inflammatory process and prevent recurrences. This report raises the possibility that VKH disease and acute myelitis share common pathogenic pathways.
Vogt-Koyanagi-Harada disease; acute myelitis; pathogenesis
To assess the utility of optical coherence tomography (OCT) in a nurse‐led, fast‐track clinic for new age‐related macular degeneration (AMD) referrals, and to see how often indocyanine green angiography (ICGA) led to an additional diagnosis to that provided by fundus fluorescein angiography (FFA).
Retrospective audit of a consecutive series of 134 new patients referred with suspected wet AMD. When visual acuity was ⩾6/60 an OCT was performed. If the OCT was consistent with “wet” AMD, the patient underwent simultaneous FFA/ICGA. The sensitivity and specificity of this clinic was calculated. The number of additional diagnoses made using ICGA was recorded.
23/134 (17.16%) patients had OCT only and were not subsequently found to have wet AMD. FFA/ICGA was performed in 111 patients, showing wet AMD in 90 (81%) patients. OCT as used in our clinic had a sensitivity of 1 and a specificity of 0.65 for detecting wet AMD. ICGA provided additional diagnoses in 19 (14.17%) patients. ICGA detected a specific vascular abnormality in 58% of the occult lesions.
OCT proved to be an effective screening tool for wet AMD in this clinic, with excellent sensitivity and reasonable specificity. ICGA provided an additional diagnosis in a significant number of cases, but did not define a vascular abnormality in all occult cases.
To evaluate the validity of the novel and noninvasive retro-mode imaging modality of confocal scanning laser ophthalmoscopy (cSLO) for detecting the morphological features of polypoidal choroidal vasculopathy (PCV).
Prospective, observational, consecutive case series.
Twenty-six patients (29 eyes) with PCV were enrolled in this study. All patients underwent comprehensive ophthalmologic examinations and imaging studies, including retro-mode imaging, fundus autofluorescence (FAF), fundus photography, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and spectral-domain optical coherence tomography (SD-OCT). We investigated the retro-mode images and compared the results with those of SD-OCT, FFA and ICGA.
In the 29 PCV eyes, the retro-mode images clearly revealed polypoidal lesions in 27 (93.1%) eyes as well as branching vascular networks in 16 (55.2%) eyes. Others findings, including pigment epithelial detachment (PED) in 20 (69.0%) eyes, neuroretinal detachment (NRD) in 3 (10.3%) eyes, cystoid macular edema (CME) in 3 (10.3%) eyes, drusen in 4 (13.8%) eyes and minute granular changes of the retinal pigment epithelium (RPE) in 12 (41.3%) eyes, were also clearly visualized. When we compared the results with those of SD-OCT, FFA and ICGA, there was no significant difference between ICGA and retro-mode imaging for finding polypoidal lesions and (or) branching choroidal vascular networks (P>0.05). However, the rate of PED detection was significantly better with retro-mode imaging than with the ICGA (P<0.05). The differences were not statistically significant between SD-OCT and retro-mode imaging for detecting PED, NRD, CME, drusen and minute granular RPE changes (P>0.05). The differences were not statistically significant between FFA and retro-mode imaging for detecting PED, NRD, CME (P>0.05).
The novel and noninvasive retro-mode imaging by cSLO is able to clearly visualize the morphological features of PCV.
Aim: To describe the angiographic features after photodynamic therapy (PDT) with verteporfin in choroidal neovascularisation (CNV) associated both with age related macular degeneration (AMD) and pathological myopia (PM).
Methods: 36 patients affected by subfoveal CNV in AMD and 25 patients with subfoveal CNV in PM underwent an ophthalmological examination including fluorescein angiography (FA) and indocyanine green angiography (ICGA) using the IMAGEnet System. Post-PDT examinations were performed 7, 30, and 90 days later.
Results: The typical angiographic aspect after PDT for AMD related CNV was a round hypofluorescence visible both on FA and on ICGA, which included both CNV and the surrounding tissues and corresponded to the area exposed to laser light. In PM the CNV appeared hypofluorescent during the early phases and gradually became hyperfluorescent during the late phases on FA, whereas on ICGA it was detectable in its whole extension as a hyperfluorescent lesion since the early phases. Differently from AMD, there was no round hypofluorescence surrounding the CNV on FA or on ICGA. Moreover, five patients in the AMD group showed hot spots on ICGA, which spontaneously disappeared during the follow up. Classic and occult components of the AMD related CNV revealed a different angiographic response to PDT, showing with the latter only a partial closure 1 week after PDT followed by a complete reopening at the first month in 100% of cases.
Conclusion: The post-PDT hypofluorescence typical of AMD related CNV, especially visible on FA, might be secondary to a combination of choriocapillary occlusion and masking effect due to swelling of retinal pigment epithelium cells. Hot spots in the AMD affected patients could be interpreted as the expression of a non-thermal choroidal vasculitis secondary to PDT.
choroidal neovascularisation; age related macular degeneration; myopia; photodynamic therapy
To report a case of peripheral polypoidal choroidal vasculopathy (PCV) which was treated successfully.
Interventional case report. Best-corrected visual acuity measurements (BCVA), slit-lamp examination, fundus biomicroscopy, fluorescein angiography (FFA), and indocyanine green angiography (ICGA) were performed at baseline examination and during the follow-up period. The patient underwent ICGA-guided argon laser to treat the active polyps.
An 82-year-old Caucasian man presented complaining of sudden deterioration of peripheral vision in his left eye (LE). His previous ocular history was associated with advanced age-related macular degeneration (AMD) involving both eyes (BE). Fundus examination revealed macular scars in BE and a large hemorrhagic pigment epithelial detachment (PED) temporal to the macula in the LE. ICGA revealed active polyps at the margins of the PED. The patient underwent ICGA-guided argon laser to treat the active polyps. Six months post-laser, the patient regained his peripheral vision with resolution of the hemorrhagic PED and remains stable until now, one year after treatment.
Appropriate treatment and regular follow-up is important in patients with PCV and peripheral lesions even if central vision is lost.
argon laser; peripheral vision; polypoidal choroidal vasculopathy
To determine the frequency of occurrence of limited clinical features which distinguish patients with Vogt-Koyanagi-Harada (VKH) disease from those with non-VKH uveitis.
Comparative case series.
1147 total patients.
All patients with bilateral ocular inflammatory disease presenting to any of ten uveitis centers in the three month period between 2006-January-01 and 2006-March-31 (inclusive) were asked to participate. The clinical and historical features of disease were obtained from the participants via direct interview and chart review. Patients were stratified based on whether they were diagnosed with VKH disease or non-VKH uveitis for statistical analysis.
Main Outcome Measures
Presence or absence of various clinical features in the two populations.
Of 1147 patients, 180 were diagnosed with VKH disease and 967 with non-VKH uveitis. Hispanics and Asians were more likely to be diagnosed with VKH than non-VKH disease compared to other ethnicities. In acute disease, the finding of exudative retinal detachment was most likely to be found in VKH disease with a positive predictive value (PPV) of 100 and negative predictive value (NPV) of 88.4, while in chronic disease, sunset glow fundus was most likely to be found, with a PPV of 94.5 and NPV of 89.2.
Numerous clinical findings have been described in the past as important in the diagnosis of VKH. The current study reveals that of these, two are highly specific to this entity in an ethnically and geographically diverse group of patients with non-traumatic bilateral uveitis. These clinical findings are 1. exudative retinal detachment during acute disease and 2. sunset glow fundus during the chronic phase of the disease.
The aim of this study was to clarify the characteristic findings in myopic choroidal neovascularization (CNV) and the relationship with lacquer crack (LC).
In all, 66 consecutive myopic CNV patients treated with photodynamic therapy and/or intravitreal anti-vascular endothelial growth factor injection in one eye were reviewed. Data from fluorescein angiography (FA) and indocyanine green angiography (ICGA), obtained simultaneously using the Heidelberg retina angiograph 2 (HRA2), were analyzed.
LCs were associated with a relatively large extent (≥3000 μm) of peripapillary choroidal atrophy and a dark rim, the proliferation of retinal pigment epithelial cells surrounding the neovascular membrane was accompanied by a small extent. Myopic CNV usually developed in the LC area surrounded by tiny crack fragments. In all, 35 patients with LCs received FA and ICGA at least twice during follow-up. LC progression was observed in nine (25.7%) treated eyes and six (23.1%) non-CNV fellow eyes. Crack fragments progressed in three distinct forms such as elongation, branching, or bridging pattern. Newly diagnosed myopic CNV was reported in 18 treated eyes and 3 fellow eyes. Progression of LCs and development of CNV occurred simultaneously in eight eyes. By multivariate Cox's regression, a statistically significant association was observed between recurrence of myopic CNV and the absence of a dark rim on ICGA.
The HRA2 instrument affords detailed high-resolution images of FA and ICGA. Notably, recurrence of myopic CNV developed in areas surrounded by new small crack fragments and LCs are considered to be important in the development of myopic CNV.
indocyanine green angiography; lacquer crack; myopic choroidal neovascularization
In this retrospective report, we present two cases of unilateral Vogt-Koyanagi-Harada (VKH) disease. These patients were evaluated with clinical, ophthalmological and laboratory examinations. Their response following corticosteroid administration was evaluated. Both patients had the characteristic clinical features of VKH involving only one eye, including disc edema, choroidal striae, multiple sub retinal yellow lesions and exudative retinal detachment. These cases indicate that the clinical and angiographic features were typical of VKH disease despite the unilateral involvement.
Choroidal Striae; Disc Edema; Fundus Fluorescein Angiography; Ultrasonography; Vogt-Koyanagi-Harada Disease
Vogt-Koyanagi-Harada (VKH) disease and sympathetic ophthalmia (SO) are two distinct entities that share common clinical and histopathological features; however, it remains unknown whether they have a common genetic susceptibility. Several studies have shown an association of human leukocyte antigen (HLA)-DR4 with VKH disease in patients of different ethnic backgrounds. We present in this paper the HLA-DRB1 genotyping analysis of a large cohort of VKH patients from southern India and compare these patients to patients with SO and to healthy individuals from the same geographic area.
VKH patients were diagnosed according to the revised criteria of the International Committee on VKH disease. Patients with granulomatous uveitis after ocular trauma or multiple eye surgeries were diagnosed as having SO. Genomic DNA was extracted from all patients and controls. Samples were analyzed for HLA-DRB1 alleles by reverse polymerase chain reaction (PCR) sequence-specific oligonucleotide (SSO) hybridization on microbeads, using the Luminex technology, and by PCR sequence-specific primers (SSP) typing for DRB1*04 allele determination. Strength of associations was estimated by odds ratios (OR) and 95% confidence intervals (CI) and frequencies were compared using the Fisher’s exact test.
HLA-DRB1 alleles were determined in 94 VKH patients, 39 SO patients, and 112 healthy controls. HLA-DRB1*04 frequency was higher in VKH patients (20.2% versus 10.3% in controls; OR=2.2, p=0.005, pc=0.067). This association was lower than the association of HLA-DRB1*04 frequency in cohorts of patients from different origins. No significant DR4 association with SO was detected. HLA-DRB1*0405 and HLA-DRB1*0410 alleles were significantly increased in VKH patients (8.5% versus 0.9% in controls; OR=10.3, 95% CI=2.34–45.5, p<0.001). These two alleles share the epitope S57-LLEQRRAA (67–74) in the third hypervariable region of the HLA-DR molecule. None of the DRB1 alleles was significantly associated with SO.
Based on the association of HLA-DRB1*0405 and HLA-DRB1*0410 alleles with VKH disease, we propose that the epitope S57-LLEQRRAA (67–74) in the third hypervariable region of the HLA-DRβ1 molecule is the relevant susceptibility epitope. This genetic component seems specific to VKH disease since no correlation could be identified in SO patients. The weaker association with HLA-DR4 in this VKH patient cohort compared to VKH patients from northern India is probably related to the lower frequency of HLA-DRB1*0405 in our study group. The HLA-DRB1 association with susceptibility to VKH syndrome seems weaker in Indian patients compared to Japanese or Hispanic patients, suggesting a different non-HLA immunogenetic background in Indian VKH patients.
To evaluate peripapillary acute zonal occult outer retinopathy in a 67-year-old man.
Images were obtained using fundus photography, fluorescein angiography (FA) and indocyanine green angiography (ICGA), fundus autofluorescence (FAF) imaging, and optical coherence tomography (OCT). Visual field testing and multifocal electroretinogram (mfERG) were also performed to evaluate retinal function.
Fundus photographs showed subtle pigmentary changes in the peripapillary region, while FAF imaging showed clearly defined hypofluorescent areas in the peripapillary region. Intense hyperfluorescent lesions were also seen underneath hypofluorescent areas. A transmission defect with a granular hyperfluorescence was visible on FA, and ICGA showed hypofluorescence within the lesion. The outer border of the peripapillary zone appeared to block the underlying choroidal fluorescence. The photoreceptor inner segment/outer segment line was absent on OCT images from this area in both eyes, and hyperreflective punctate drusen-like materials were present at the outer border of the peripapillary zone. Visual field testing by Goldmann perimetry showed blind-spot enlargement, and mfERG showed corresponding amplitude reductions.
We speculate that the intense hyperautofluorescent material at the outer border of the peripapillary zone might be accumulated drusenoid lipofuscin.
Acute zonal occult outer retinopathy; Fundus autofluorescence imaging; Fluorescein angiography; Indocyanine green angiography; Optical coherence tomography; Visual field; Multifocal electroretinogram
To assess the effects of photodynamic therapy (PDT) with verteporfin in the treatment of subfoveal choroidal neovascularisation (CNV) secondary to Vogt‐Koyanagi‐Harada disease (VKH).
Six eyes of six patients with VKH who developed subfoveal CNV underwent standard PDT. Repeated treatments were performed at 3 month intervals for persistent leakage. Charts and angiographic data were analysed retrospectively.
Age of patients ranged between 17 years and 27 years. Five CNV lesions were recent and classic (greatest lesion diameter was 1100–3100 μm). One CNV was chronic and partially scarred. Mean visual acuity (VA) at presentation was 20/200. Five patients had more than 1 year of follow up. In five eyes there was active inflammation and CNV. Of these eyes, the first three required one PDT each. The final CNV scar was smaller/stable with improvement of VA in two eyes. The third developed a larger CNV scar with loss of two lines of VA. Submacular fibrosis developed in all three. In the fourth eye, mild CNV leakage persisted after one PDT but hazy media precluded a second PDT. At 18 months the CNV scar and VA were stable. The fifth case, with mild inflammation, required three PDT. The CNV leakage became minimal, the lesion became smaller, and VA improved significantly. The sixth eye with CNV had no inflammation and needed two PDT sessions to halt the CNV leakage. The final lesion was smaller and vision was stable. There were no PDT related complications in our series.
Photodynamic therapy with verteporfin appears to be a safe and viable treatment option for subfoveal CNV secondary to VKH. It offers a chance for stabilisation or even improvement of vision. Further study is warranted.
photodynamic therapy; subfoveal choroidal neovascularisation; Vogt‐Koyanagi‐Harada disease
We report a case of multiple evanescent white dot syndrome (MEWDS) that presented with putative idiopathic choroidal neovascularisation (ICNV) before showing angiographic signs typical of MEWDS. A 16-year-old male presented with unilateral metamorphopsias and visual loss in his left eye. ICNV with subretinal hemorrhage was diagnosed and treated with intravitreal Avastin®. Fifteen days later, regression of choriodal neovascularization (CNV) was documented together with the appearance of fluorescein angiography (FA) and indocyanine green angiography (ICGA) signs typical for MEWDS, that included faint mottled FA hyperfluorescence in the mid-peripheral fundus, irregularly shaped mid-peripheral ICGA dark areas in the intermediate angiographic phase that were clearly delineated in the late phase as well as peripapillary hypofluorescence. Fundus examination appeared completely normal during the follow-up except for the CNV hemorrhage noted at the initial visit. This case demonstrates the need to consider ICNV as a diagnosis of exclusion until inflammatory causes have been eliminated. In this case, the underlying occult inflammatory condition would have been missed without the ICGA data that clearly showed signs of MEWDS that was supported by FA findings.
Idiopathic Choroidal Neovascularization; Indocyanine Green Angiography; Multiple Evanescent White Dot Syndrome