Birdshot Lesions Research Articles

Choroidal Findings in Eyes With Birdshot Chorioretinitis Using Enhanced-Depth Optical Coherence Tomography.

The purposes of this study were to describe choroidal findings observed using optical coherence tomography with enhanced depth imaging (EDI-OCT) in eyes with birdshot chorioretinitis (BSCR) and to test the hypothesis that these findings are related to participant demographics, clinical characteristics, and treatment. In a multicenter, cross-sectional study, 172 eyes of 86 individuals with BSCR underwent a standardized clinical evaluation, including defined protocols for EDI-OCT imaging, with macular and peripapillary volume scans. Choroidal findings were compared to demographic information, ophthalmic examination findings, and treatment history, using logistic regression models. EDI-OCT images were evaluated by two independent, masked graders. Median age was 56 years old; 54 participants (62.8%) were female. One or more choroidal lesions (a predefined hyporeflective zone) were identified in 105 eyes (63.6%). Median choroidal thickness was 293 μm. Choroidal lesions were associated with longer disease durations (odds ratio [OR]: 1.08; P = 0.03), increased vitreous haze (>0.5+; OR: 4.43; P = 0.02), presence of macular edema (OR: 3.00; P = 0.02), and thick choroids (OR: 3.89; P = 0.001). Use of immunomodulatory therapy was associated with lower risk of thin choroids (lower 25th percentile, OR: 0.17; P = 0.001) or thick choroids (upper 25th percentile, OR: 0.22; P = 0.002). At least some choroidal lesions did not have corresponding, clinically apparent "birdshot lesions" on fundus examination. Choroidal abnormalities identified by EDI-OCT imaging are common in the macular and peripapillary regions of eyes with BSCR. Choroidal lesions were associated with clinical signs of inflammation, suggesting that they represent foci of disease activity. EDI-OCT may provide useful information about disease mechanisms and response to treatment in future, longitudinal studies of BSCR.

RETINAL AND CHOROIDAL VASCULATURE IN BIRDSHOT CHORIORETINOPATHY ANALYZED USING SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

To describe retinal and choroidal vascular changes in eyes with birdshot chorioretinopathy using optical coherence tomography angiography. Patients underwent imaging using the AngioVue prototype software of the RTVue XR spectral domain optical coherence tomography device (Optovue, Inc) between September and December 2014. Two trained patients evaluated the optical coherence tomography angiography images for changes in the retinal and choroidal vasculature in the posterior pole. Four of eight eyes (50%) had birdshot lesions in the posterior pole as demonstrated on fundus photography. All of these eyes demonstrated the areas of decreased choroidal blood flow below the disrupted retinal pigment epithelium. Larger choroidal vessels bordered the birdshot lesions. All eyes analyzed showed retinal thinning, telangiectatic vessels, and an increased intercapillary space. Capillary dilatations and loops were each seen in 7 of 8 eyes (88%). Optical coherence tomography angiography provides precise microvascular detail of the retinal vasculature and choriocapillaris that allows for the noninvasive visualization of the birdshot lesions and changes in the inner retina. The optical coherence tomography angiography images delineated widespread retinal vascular findings not previously described in the literature. In the future, optical coherence tomography angiography could be a useful tool to evaluate the natural history of birdshot chorioretinopathy, its progression, and the effect of treatment in these patients.

Diagnostic Sensitivity of Indocyanine Green Angiography for Birdshot Chorioretinopathy

To describe a cohort of patients with birdshot chorioretinopathy who did not manifest birdshot lesions on clinical examination but had retinal vasculitis, low-grade to moderate vitritis, and hypocyanescent lesions on indocyanine green angiography (ICGA). Case series of 3 patients with mild to moderate vitritis and retinal vasculitis without definite birdshot lesions on clinical examination evaluated from January 2007 to December 2014 at 4 academic ophthalmology centers. All patients' results were positive for human leukocyte antigen-A29. All cases had hypocyanescent lesions visible on ICGA but not detectable on fluorescein angiography. Patients with retinal vasculitis and low-grade vitritis with or without macular edema may have birdshot chorioretinopathy evident on ICGA before lesions are visible on clinical examination or fluorescein angiography. Expanding birdshot chorioretinopathy diagnostic criteria to include the presence of hypocyanescent lesions on ICGA could improve the sensitivity of diagnosis.

Early diagnosis and treatment changes disease phenotype in birdshot retinochoroiditis

AbstractBackground and Aim: The diagnosis of birdshot retinochoroiditis (BRC) relies mainly on the presence of oval‐shaped "birdshot lesions" (BL) part of the currently used inappropriate diagnostic criteria that do not use diagnostic tools for choroidal disease such as ICG angiography (ICGA) and do not allow to make an early diagnosis. It is now well‐known that when BL become apparent, the disease has already been evolving since months or years. Patients and methods: Using appropriate diagnostic criteria, including vitritis and retinal vasculitis in at least one eye, presence of ICGA detected choroiditis in both eyes and the presence of the HLA‐A29 antigen, we compared a group of patients treated early (< 10 months of symptoms) with a group of patients treated after more than 10 months of symptoms. Mean follow‐up was 10 years in bothe groups. Results: Patients treated early almost never developed the characteristic BL, whereas patients treated late all presented BL at start of therapy that almost never disappeared after treatment. Conclusion: Early diagnosis and treatment modifies the phenotype of BRC by preventing the apparition of BL clearly indicating that the presently used diagnostic criteria are completely inappropriate.

Early and sustained treatment modifies the phenotype of birdshot retinochoroiditis

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

Early and sustained treatment modifies the phenotype of birdshot retinochoroiditis

AbstractThe present appraisal of Birdshot retinochoroiditis (BCR) is based on obsolete criteria that only represent advanced disease. Early diagnosis of BC can only be done using indocyanine green angiography (ICGA) showing the typical choroidal involvement. The aim of this study was to investigate long‐term follow ups of BRC patients regarding the impact of early treatment on the phenotype of BRC, both on ICGA findings as well as fundus appearance. 13 patients had sufficient data to be included in the study. Out of 13 patients, 8 showed typical birdshot lesions at presentation according to the presently used criteria. A significant proportion of the “no lesions” group did not develop typical birdshot lesions throughout the course. Mean treatment delay was 45.50 ± 33.39 months in the “typical lesions” group and 5.80 ± 2.95 months in the “no lesions” group. Early treatment can avoid the apparition of typical BRC lesions in the same fashion as early and heavy treatment can avoid the development of “sunset glow” fundus in Vogt‐Koyanagi‐Harada disease (VKH). Early diagnosis relies heavily on ICGA, that together with FA, visual field testing and presence of HLA‐A29 antigen allows the diagnosis before typical birdshot lesions can be seen.

Indocyanine Green Angiography (ICGA) is Essential for the Early Diagnosis of Birdshot Chorioretinopathy

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. 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. 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. 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.

Bilateral choroidal neovascularization in birdshot retinochoroidopathy treated with intravitreal injections of triamcinolone and bevacizumab.

To describe a patient with birdshot retinochoroidopathy (BRC) with bilateral choroidal neovascularization (CNV) who was treated with intravitreal injection of bevacizumab and antiinflammatory medications. Interventional case report. A 35-year-old woman with bilateral CNV associated with BRC. The patient was treated with intravitreal injection of triamcinolone, photodynamic therapy, and intravitreal injection of bevacizumab in one eye, while the fellow eye was treated with intravitreal injection of triamcinolone. Immunosuppressive therapy was performed in the course of the treatment. Not only did the neovascularization respond, but the birdshot lesions vanished as well. BRC can have secondary CNV that, as in the current case, responds favorably to treatment. We serendipitously observed regression of the choroidal inflammatory lesions with intravitreal injection of triamcinolone.

Fundus Autofluorescence in Birdshot Chorioretinopathy

To investigate the characteristics of fundus autofluorescence in birdshot chorioretinopathy (BSCR). Retrospective, observational case series. Sixteen eyes of 8 consecutive patients with BSCR (3 men, 5 women). Color and autofluorescence photography and optical coherence tomograms of patients with BSCR seen in a referral practice were evaluated. Autofluorescent characteristics in BSCR. The 8 patients ranged in age from 35 to 73 years (mean, 56.9 years). Of the 16 eyes, 11 eyes (69%) of 6 patients had retinal pigment epithelium (RPE) atrophy as evidenced by hypoautofluorescent regions. Some of the hypoautofluorescent areas corresponded to the hypopigmented birdshot lesions, but the others did not necessarily show a correspondence. Eight eyes (50%) of 4 patients showed linear hypoautofluorescent streaks along the retinal vessels, most of which corresponded to visible changes at the level of the RPE. Placoid hypoautofluorescence in the macula was seen in 6 eyes (38%) of 3 patients and was correlated significantly with best-corrected visual acuity of 20/50 or less (P<0.05). Autofluorescence photography demonstrated the RPE atrophy, which was hard to see by other means of investigation. The areas of RPE atrophy did not necessarily correspond to the hypopigmented lesions, which suggested that both the choroid and the RPE can be affected independently. Retinal pigment epithelium atrophy in the macula may be an important cause of poor central visual acuity in eyes with BSCR.

Research Criteria for the Diagnosis of Birdshot Chorioretinopathy: Results of an International Consensus Conference

To present and evaluate research criteria for the diagnosis of birdshot chorioretinopathy. Consensus statement. Investigators who attended an international conference on birdshot chorioretinopathy drafted diagnostic criteria, which were refined after comment by conference attendees. Sensitivity and specificity of the criteria were evaluated by review of 82 previously diagnosed cases and evaluation of 80 consecutive patients with other forms of uveitis. Criteria, including presence of "birdshot lesions" and mild intraocular inflammation in both eyes, are based on clinical findings in the absence of systemic disease; HLA-A29 is supportive of diagnosis, but not required. Diagnostic criteria were found to be 100% specific and 97.5% sensitive. Positive predictive value was 100%; negative predictive value was 97.6%. Standardized criteria for the diagnosis of birdshot chorioretinopathy will facilitate clinical research and the comparison of results from studies performed at different institutions by ensuring a homogeneous population of study patients.

Longitudinal Cohort Study of Patients With Birdshot Chorioretinopathy. I. Baseline Clinical Characteristics

To describe baseline clinical characteristics of a cohort of 80 patients with birdshot chorioretinopathy in anticipation of a longitudinal study, and to identify relationships between visual acuity, symptoms, and ophthalmic findings. Single-center cross-sectional study. A standardized examination was performed in the same order on a single day for each patient. A grading system for birdshot lesions was established prospectively to evaluate the following lesion characteristics: quantity, distribution, morphology, and pigmentation. Relationships between clinical features of disease were sought in multivariate analyses that adjusted for age, duration of uveitis, and treatment. Mean age at baseline examination was 55.6 years. Median best-corrected visual acuity (BCVA) was 0.8 (range, counting fingers to 1.2). There were no relationships between BCVA and any birdshot lesion characteristic. The most common cause of BCVA < or =0.4 was macular edema. Visual symptoms were present in 78 patients (97.5%), including 17 (94.4%) of 18 patients with BCVA > or =1.0 in both eyes. Blurred vision was associated with decreased BCVA (P = .02) and macular edema (P = .022). Increased lesion pigmentation was associated with complaints of blurred vision (P = .030), vibrating vision (P = .011), and nyctalopia (P = .056). Symptoms are common in patients with birdshot chorioretinopathy, even among those with good BCVA. Lesion pigmentation may be a marker of decreased visual function that is not reflected in central visual acuity. These findings highlight the limitation of using visual acuity measurements for monitoring patients with birdshot chorioretinopathy and as an outcome measure for studies of this disease.

Choroidal abnormalities in birdshot chorioretinopathy: an indocyanine green angiography study.

Birdshot chorioretinopathy is a rare inflammatory disorder with an insidious onset that can slowly progress to severe visual loss. The pathogenesis is unknown. This study used indocyanine green (ICG) angiography to investigate the degree of choroidal vascular involvement with progression of disease and to determine the nature of the birdshot lesions. Seven patients with birdshot chorioretinopathy had ICG angiography performed with a scanning laser ophthalmoscope at various stages of clinical disease. Results were compared with fluorescein fundal angiography (FFA). All large choroidal vessels appeared normal. The birdshot lesions were demonstrated with ICG but not with FFA and were represented by dark areas on ICG angiography. Typically these areas were bordered by large or medium-sized choroidal vessels and their appearance suggested small choroidal vessel hypoperfusion. In disease of recent onset, some lesions masked fluorescence from large underlying choroidal vessels possibly due to inflammatory choroidal infiltrates. In long-standing disease, the choroidal angioarchitecture was relatively normal within the birdshot lesions. This study of birdshot chorioretinopathy demonstrates abnormalities in the small choroidal vessels within the birdshot lesions. ICG angiography detects the birdshot lesions more readily than FFA and may be of benefit in assessing disease activity.