Do not discount the diagnosis of VKH based on race: self-reported race and ethnicity of patients with Vogt-Koyanagi-Harada disease in a predominantly white population.

BACKGROUND: We examined the racial and ethnic distribution of patients with Vogt-Koyanagi-Harada disease (VKH) in a Midwestern US population through a retrospective chart review of patients with VKH seen in a tertiary referral centre between 2012 and 2017. All patients were diagnosed by one uveitis specialist (DAG). We identified 32 patients with VKH seen during this time period. The mean age at diagnosis was 37.7 ± 15.7 years, 7 were male, 25 female. Mean follow-up was 36.7 ± 21.7 months. Nine patients reported themselves as White non-Hispanic, (28.1%), 9 as Black/African-American (28.1%), 2 as Asian (6.3%) and 9 as Hispanic or Latino (28.1%). Three patients (9.4%) were of Middle-Eastern origin. The 2010 census results for race and ethnicity in the state of Illinois were: 71.5% White, 14.5% Black/African-American, 4.6% Asian, and 6.7% as Some Other Race. From the total population 15.8% reported themselves as Hispanic or Latino (of any race). CONCLUSIONS: VKH was much more frequent among white non-Hispanic patients (28.1%) and Black/African-American patients (28.1%) in our patient population than in previous reports from the US (3-14% and 4-23% respectively). While Hispanic patients in this series were over represented in the VKH population compared with the overall census data, the percentage of VKH patients in this series who were White non-Hispanic and Hispanic was the same. The diagnosis of VKH should be considered in any patient with the appropriate clinical features, regardless of race or ethnicity.

The Role of Screening for Asymptomatic Ocular Inflammation in Sarcoidosis.

PURPOSE: To determine the utility of routine screening ophthalmic exam in patients with systemic sarcoidosis and no history of uveitis. METHODS: Prospective, single-center, observational study conducted at Northwestern University from October 11, 2012 to October 1, 2020 of new patients with biopsy-proven systemic sarcoidosis and no history of uveitis, referred by medical subspecialists for screening ophthalmic exam. RESULTS: Forty-nine patients, with mean age of 51 ± 8.7 years, 59% female, 47% African American, 43% Caucasian, were enrolled. The majority (55%) had no ocular symptoms. The most common location of ocular involvement was the adnexa, in the form of conjunctival nodules (62%) and aqueous tear deficiency (23%). Intraocular inflammation was detected in 6 patients (13%); only 2 had active disease requiring treatment (4%). No asymptomatic patient had ocular involvement necessitating treatment. CONCLUSION: Screening exams are indicated in sarcoidosis patients with ocular symptoms. No benefit of screening was demonstrated in asymptomatic patients.

Tubulointerstitial Nephritis and Uveitis Syndrome: Characterization of Clinical Features.

Purpose: To describe clinical manifestations of patients with tubulointerstitial nephritis and uveitis syndrome (TINU) with a focus on posterior segment findings.Methods: Retrospective chart review.Results: 17 patients were diagnosed with TINU. Ten (59%) were female. Average age at presentation was 19 years (range 7-49). Urine ß2-microglobulin was elevated in all tested patients. Six patients (35%) had isolated anterior uveitis. Eleven (65%) had posterior segment findings. Three (18%) had multifocal choroidal lesions, seven (41%) had disc edema, four (24%) had macular edema, three (18%) had choroidal neovascular membranes, two (12%) had retinal vasculitis, one (6%) had disc neovascularization and vitreous hemorrhage, one (6%) had bilateral posterior scleritis. Eleven (65%) required systemic steroid therapy, seven (41%) required immunomodulatory therapy.Conclusions: TINU occurs in all age groups. Posterior segment manifestations are common. Most patients required systemic steroid therapy, with a significant number requiring long-term systemic immunomodulation. Careful fundus examination is required on all patients with TINU.

Partial Scleral Rupture Presenting 4 Years After Laser in Situ Keratomileusis.

PURPOSE: To report a case of partial scleral rupture after laser in situ keratomileusis (LASIK) surgery. METHODS: This is a retrospective case report describing a late complication of LASIK surgery. RESULTS: A 32-year-old white woman with a history of LASIK surgery performed with a mechanical microkeratome 4 years previously presented for evaluation of partial bilateral scleral ruptures. An arcuate filtering bleb along the contour of the cornea was found on the right eye with associated hypotony and corneal astigmatism. There were no signs or symptoms of prior inflammation, and rheumatologic workup was negative. Imaging revealed scleral microbreaks in the absence of adjacent thinning. CONCLUSIONS: The pattern and location of thinning without findings consistent with inflammatory disease suggest that the partial bilateral scleral rupture was because of mechanical damage at the time of LASIK, possibly compounded by the hormonal changes of pregnancy.

Tuberculous Uveitis Presenting with a Bullous Exudative Retinal Detachment: A Case Report and Systematic Literature Review.

Purpose: To describe tuberculous uveitis (TU) presenting as a bullous retinal detachment (RD) and to perform a comprehensive literature review on TU with similar features. Methods: Observational case report and systematic literature review. Results: An 84-year-old woman presented with bilateral granulomatous uveitis and bullous RD in the left eye. The interferon gamma release assay was strongly positive, but all other tests were unremarkable. The patient was diagnosed with TU and started on anti-tubercular therapy (ATT) and systemic steroids with excellent treatment response. Twenty-six articles (32 cases) reported TU with exudative RD. Choroidal tuberculoma was the most common clinical manifestation, followed by optic disc edema and retinal exudate. Systemic steroids with ATT improved vision in more patients (78.6%) than ATT alone (50.0%) or oral steroids followed by ATT (50.0%). Conclusion: Atypical presentations of TU make diagnosis and treatment difficult. A high level of suspicion for TU is needed to minimize ocular morbidity.

What is new in paediatric uveitis?

PURPOSE OF REVIEW: The aim of this study was to summarize the demographics and causes of paediatric uveitis and to discuss the latest treatment options for noninfectious paediatric uveitis. RECENT FINDINGS: In the last few years, reports out of Europe, Asia and the Americas have shown that the causes of paediatric uveitis can vary greatly geographically. With ongoing research into therapeutics, particularly the development of biologic agents, the treatment of noninfectious entities has evolved over time. SUMMARY: Epidemiology data show that most paediatric uveitis cases worldwide are chronic and have noninfectious causes. Fortunately, we have more treatment options than ever to combat these sight-threatening diseases.

Visualization of Photoreceptors in Birdshot Chorioretinopathy Using Adaptive Optics Scanning Laser Ophthalmoscopy: A Pilot Study.

PURPOSE: Adaptive optics scanning laser ophthalmoscopy (AOSLO) allows en face visualization of specific layers of the retina. This pilot study evaluated the ability of AOSLO to visualize photoreceptor integrity in patients with birdshot chorioretinopathy (BCR). METHOD: A total of 16 consecutive patients with HLA-A29+ BCR were imaged using the prototype Apaeros retinal imaging system. Images of high quality were aligned with infrared reflectance photos and correlated with spectral domain optical coherence tomography (SD-OCT). RESULTS: Images of four eyes of three patients were of sufficient quality to allow posterior pole montage and point-to-point correlation with SD-OCT. Areas of photoreceptor disruption on SD-OCT were seen as patchy areas of loss on AOSLO, whereas areas of intact interdigitation zone and inner segment/outer segment junction correlated with normal appearing photoreceptors on AOSLO. CONCLUSIONS: Using AOSLO, we found one instance of subclinical photoreceptor disruption not seen on SD-OCT. Ultimately, there are unique challenges associated with imaging BCR patients using AOSLO.

Poststreptococcal syndrome presenting as posterior scleritis in a child.

Posterior scleritis in children is very rare. In contrast to the adult form, pediatric posterior scleritis has not previously been associated with any systemic disorder. We describe a case of an 11-year-old girl who presented with left eye pain and redness and was found to have posterior scleritis on ultrasonography. Her laboratory work-up revealed a highly elevated antistreptolysin O titer; the rest of her serologic and radiologic evaluation was unremarkable. She was diagnosed with presumed poststreptococcal posterior scleritis and improved with a slow taper of oral steroids.

Chronic postoperative endophthalmitis after cataract surgery secondary to vancomycin-resistant Ochrobactrum anthropi: case report and literature review.

BACKGROUND: The aim of this study was to report an unusual case of chronic postoperative endophthalmitis following cataract surgery, secondary to Ochrobactrum anthropi that was found to be resistant to vancomycin. FINDINGS: Anterior chamber paracentesis cultures grew gram negative bacilli Ochrobactrum anthropi. The patient was treated with a series of intracameral injections of moxifloxacin, with adjuvant oral moxifloxacin. Posterior sub-Tenon and oral corticosteroids were used to treat cystoid macular edema. Explantation of the intraocular lens (IOL)-capsular bag complex was avoided. CONCLUSIONS: Chronic postoperative endophthalmitis is a rare entity, often due to indolent pathogens that sequester in the capsular bag. Aggressive surgical intervention may be avoided with the use of adequate intraocular antibiotic, provided that the offending organism demonstrates appropriate antibiotic susceptibilities.

Developing SDOCT to assess donor human eyes prior to tissue sectioning for research.

BACKGROUND: To compare spectral domain optical coherence tomography (SDOCT) cross-sectional images of human central retina obtained from donor eyes with and without age-related macular degeneration (AMD) to corresponding histopathology from light micrographs. To establish the utility of SDOCT for localizing pathology in the posterior eyecup, for identifying ocular disease in donor eyes, or for directing subsequent sectioning of retinal lesions for research. METHODS: Seven consecutive human donor eyes were selected based on age. The eyes, with the anterior segment removed, were imaged by SDOCT with a focusing aspheric lens. Four eyes were from donors with a clinical history of AMD, and three were from age-matched donors with no history of AMD. Histopathological correlation of morphological changes detected in three eyes by SDOCT was obtained for comparison to step serial-sectioned light microscopy images of the formalin-fixed, paraffin-embedded retina. A simplified imaging setup was tested on an enucleated porcine eye for comparison. RESULTS: AMD pathology was detected and localized in four eyes by SDOCT. The SDOCT images correlated with the histopathology observed by light microscopy in each sectioned eye. Pathologies included a subfoveal neovascular lesion with subretinal fluid, peripapillary neovascularization, epiretinal membrane, foveal cyst, choroidal folds, and drusen. Similar imaging was possible with the simplified setup. CONCLUSIONS: SDOCT imaging identified retinal disease of the posterior eyecup in human donor eyes. Pathology detected with SDOCT was verified by light microscopy in three eyes, supporting the utility of SDOCT as a screening tool for research.

Photoreceptor layer thinning over drusen in eyes with age-related macular degeneration imaged in vivo with spectral-domain optical coherence tomography.

PURPOSE: Detect changes in the neurosensory retina using spectral-domain optical coherence tomography (SD OCT) imaging over drusen in age-related macular degeneration (AMD). Quantitative imaging biomarkers may aid in defining risk of disease progression. DESIGN: Cross-sectional, case-control study evaluating SD OCT testing in AMD. PARTICIPANTS AND CONTROLS: Seventeen eyes of 12 subjects with nonneovascular AMD and drusen and 17 eyes of 10 age-matched control subjects. METHODS: Spectral-domain OCT imaging across the fovea in the study eye with multiple 10- to 12-mm scans of 1000 A scans each. MAIN OUTCOME MEASURES: In summed SD OCT scans, the height of individual retinal layers either over drusen or at corresponding locations in the control eye and qualitative changes in retinal layers over drusen. Secondary measures included photoreceptor layer (PRL) area, inner retinal area, and retinal pigment epithelium (RPE)/drusen area. RESULTS: The PRL was thinned over 97% of drusen, average PRL thickness was reduced by 27.5% over drusen compared with over a similar location in controls, and the finding of a difference was valid and significant (P=0.004). Photoreceptor outer segments were absent over at least 1 druse in 47% of eyes. Despite thinning of the PRL, inner retinal thickness remained unchanged. We observed 2 types of hyperreflective abnormalities in the neurosensory retina over drusen. Distinct hyperreflective speckled patterns occurred over drusen in 41% of AMD eyes and never in control eyes. A prominent hyperreflective haze was present in the photoreceptor nuclear layer over drusen in 67% of AMD eyes and more subtly in the photoreceptor nuclear layer in 18% of control eyes (no drusen). CONCLUSIONS: With SD OCT as used in this study, we can easily detect and measure changes in PRL over drusen. Decreased PRL thickness over drusen suggests a degenerative process, with cell loss leading to decreased visual function. The hyperreflective foci overlying drusen are likely to represent progression of disease RPE cell migration into the retina and possible photoreceptor degeneration or glial scar formation. A longitudinal study using SD OCT to examine and measure the neurosensory retina over drusen will resolve the timeline of degenerative changes relative to druse formation.

Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography.

OBJECTIVES: To evaluate the spectrum of foveal architecture in pediatric albinism and to assess the utility of spectral-domain optical coherence tomography (OCT) in ocular imaging of children with nystagmus. METHODS: Spectral-domain OCT imaging was performed on study subjects in 3 groups: subjects with ocular albinism (OA) or suspected OA with foveal hypoplasia, with nystagmus, and with or without iris transillumination; a subject with oculocutaneous albinism and Hermansky-Pudlak syndrome; and control subjects. Dense volumetric scans of each fovea were captured using standard and handheld spectral-domain OCT devices. Images were postprocessed and scored for the presence and configuration of each retinal layer across the fovea. RESULTS: High-quality spectral-domain OCT images obtained from each subject revealed a range of abnormalities in subjects with OA or suspected OA and the subject with oculocutaneous albinism and Hermansky-Pudlak syndrome: persistence of an abnormal, highly reflective band across the fovea, multiple inner retinal layers normally absent at the center of the fovea, and loss of the normally thickened photoreceptor nuclear layer at the fovea when compared with that in control subjects. The optic nerve was elevated in multiple eyes of subjects with OA or suspected OA and the subject with oculocutaneous albinism and Hermansky-Pudlak syndrome. CONCLUSIONS: A spectrum of foveal morphological abnormalities is seen in subjects with OA or suspected OA, which in some cases contrasted with previous studies using time-domain OCT. These OCT findings clarify the morphology of foveal hypoplasia seen clinically. This imaging modality may be useful in evaluating children.

Drusen ultrastructure imaging with spectral domain optical coherence tomography in age-related macular degeneration.

PURPOSE: To categorize drusen ultrastructure in age-related macular degeneration (AMD) using spectral domain optical coherence tomography (SDOCT) and correlate the tomographic and photographic drusen appearances. DESIGN: Prospective case series. PARTICIPANTS: Thirty-one eyes of 31 patients with non-neovascular AMD. METHODS: Subjects with drusen and a clinical diagnosis of AMD were enrolled in an SDOCT imaging study from August of 2005 to May of 2007. Foveal linear scans were acquired, and the image data were processed for analysis. Drusen were scored by 4 morphologic categories: shape, predominant internal reflectivity, homogeneity, and presence of overlying hyper-reflective foci. The prevalences of each morphologic pattern and combinations of morphologic patterns observed were calculated. The photographic appearance of each druse was compared with the tomographic classification. Interobserver and intraobserver agreement analysis was performed. MAIN OUTCOME MEASURES: Prevalence of morphologic parameters using SDOCT. RESULTS: Twenty-one eyes of 21 patients had SDOCT B-scans of adequate quality for analysis. On the basis of the above morphologic categories, 17 different drusen patterns were found in 120 total drusen. The most common was convex, homogeneous, with medium internal reflectivity, and without overlying hyper-reflective foci, present in 17 of 21 eyes (81%). Of the 16 eyes (76%) with nonhomogeneous drusen, 5 had a distinct hyper-reflective core. Hyper-reflective foci overlying drusen were in 7 eyes (33%). Although half of the photographically soft-indistinct drusen were convex with medium internal reflectivity and homogeneous without overlying hyper-reflective foci, the other half had significant variability in their tomographic appearance. Both interobserver and intraobserver agreement in drusen grading were high. Readers agreed the most when grading drusen shape and reflectivity, whereas the least agreement was for drusen homogeneity. CONCLUSIONS: Drusen ultrastructure can be imaged with SDOCT and characterized with a simple grading system. Photographic appearance may predict some but not all tomographic appearances. Trained observers have a high level of agreement with this grading system. These in vivo morphologic characteristics imaged with SDOCT may be distinct subclasses of drusen types, may relate closely to ultrastructural drusen elements identified in cadaveric eyes, and may be useful imaging biomarkers for disease severity or risk of progression. This will require validation from further studies.