A case of recalcitrant pediatric Vogt-Koyanagi-Harada disease successfully controlled with adalimumab.

Vogt-Koyanagi-Harada (VKH) disease is uncommon in the pediatric population and can have an aggressive course with serious visual sequelae. A 12-year-old Han Chinese American female, who presented with mild headaches and panuveitis with diffuse serous retinal detachments, was diagnosed with VKH. Despite treatment with a combination of high-dose systemic corticosteroids, intravitreal triamcinolone injection, and mycophenolate mofetil, ocular inflammation was inadequately controlled. Addition of adalimumab allowed for inflammation remission, improvement of vision, and tapering of systemic corticosteroids. Escalation of immunosuppression until remission appears to be critical in this population. Further research is needed to understand the complex pathophysiology of VKH and investigation for similar efficacy of other anti-tumor necrosis factor-alpha agents will need to be performed.

Optical coherence tomography evaluation in the Multicenter Uveitis Steroid Treatment (MUST) trial.

PURPOSE: To describe the evaluation of optical coherence tomography (OCT) scans in the Muliticenter Uveitis Steroid Treatment (MUST) trial and report baseline OCT features of enrolled participants. METHODS: Time-domain OCTs acquired by certified photographers using a standardized scan protocol were evaluated at a reading center. Accuracy of retinal thickness data was confirmed with quality evaluation, and caliper measurement of centerpoint thickness (CPT) was performed when retinal thickness data were unreliable. Morphological evaluation included cysts, subretinal fluid, epiretinal membranes (ERMs), and vitreomacular traction. RESULTS: Of the 453 OCTs evaluated, automated retinal thickness was accurate in 69.5% of scans, caliper measurement was performed in 26%, and 4% were ungradable. Intraclass correlation was.98 for reproducibility of caliper measurement. Macular edema (centerpoint thickness ≥ 240 µm) was present in 36%. Cysts were present in 36.6% of scans and ERMs in 27.8%, predominantly central. Intergrader agreement ranged from 78 to 82% for morphological features. CONCLUSION: Retinal thickness data can be retrieved in a majority of OCT scans in clinical trial submissions for uveitis studies. Small cysts and ERMs involving the center are common in intermediate and posterior/panuveitis requiring systemic corticosteroid therapy.

Time Domain Optical Coherence Tomography in Familial Vitreous Amyloidosis Associated Transthyretin Met30 Mutation.

Vitreous amyloidosis is characterized by progressive loss of vision from amyloid accumulation of deposits on the retina and in the vitreous. Time domain optical coherence tomography (TD-OCT) was used in a case of familial transthyretin vitreous amyloidosis Met30 mutation to image the anterior vitreous deposits, which showed high reflectivity of very different from vitreous found in unaffected individuals. TD-OCT may be a useful adjunctive test to diagnose vitreous amyloidosis when masquerade syndromes are suspected. There may be more easeful imaging in TD-OCT of vitreous abnormalities than with spectral domain OCT (SD-OCT). Further study should be performed comparing TD versus SD-OCT in families with vitreous amyloidosis and TD vitreous amyloidosis versus TD in other intermediate uveitis.

Lesões coriorretinianas em mães de crianças com toxoplasmose congênita no national collaborative Chicago-based, toxoplasmosis study / Chorioretinal lesions in mothers of children with congenital toxoplasmosis in the national collaborative Chicago-based, congenital toxoplasmosis study

Aims: To determine whether mothers of children with congenital toxoplasmosis have chorioretinal lesions consistent with toxoplasmosis. Methods: Prospective cohort study. Ophthalmologists in our study have examined 173 children with congenital toxoplasmosis in a hospital outpatient setting. These children were referred to us by their primary care physicians. One hundred and thirty mothers of these children had retina examinations of both eyes at least once. Main outcome measure was lesion(s) consistent with ocular toxoplasmosis. Results: Of 130 mothers examined between 1991-2005, 10 (7.7%, 95% Confidence Interval 3.8%, 13.7%) had chorioretinal lesions which likely represent resolved toxoplasmic chorioretinitis. Most of these were small peripheral chorioretinal lesions. None reactivated between 1991-2005. Conclusions: Chorioretinal lesions consistent with quiescent ocular toxoplasmosis occur in mothers of children with congenital toxoplasmosis in the United States.

Whole-body 18FDG PET-CT imaging of systemic sarcoidosis: ophthalmic oncology and uveitis.

PURPOSE: To describe whole-body 18-fluorodeoxyglucose (FDG) positron emission tomography/computed radiographic tomography (PET-CT) imaging of ophthalmic patients with systemic sarcoidosis. METHODS: Four systemic sarcoidosis patients were evaluated with PET-CT for staging. Two had been treated for conjunctival melanoma and two had been referred for atypical choroidal tumors. PET-CT images were studied for presence of tumor or tissue with increased standardized uptake values, indicating increased metabolic activity. RESULTS: In all cases, PET-CT revealed focal systemic lesions with increased uptake (SUV range 1.7-5.9 kg/mL). Cases 1 and 2 had a previous diagnosis of sarcoidosis (without ocular involvement), while cases 3 and 4 were diagnosed during their work-up. PET-CT revealed the presence and distribution of systemic sarcoid granulomas. CONCLUSIONS: In this series, PET-CT staged patients with eye cancer and systemic sarcoidosis and aided in differentiating between a metastatic choroidal tumor and uveal sarcoid granuloma. PET-CT offers a method to assess the presence and distribution of systemic sarcoidosis.

Cataracts in congenital toxoplasmosis.

PURPOSE: To determine the incidence and natural history of cataracts in children with congenital toxoplasmosis. METHODS: Children referred to the National Collaborative Chicago-based Congenital Toxoplasmosis Study (NCCCTS) between 1981 and 2005 were examined by ophthalmologists at predetermined times according to a specific protocol. The clinical course and treatment of patients who developed cataracts were reviewed. RESULTS: In the first year of life, 134 of 173 children examined were treated with pyrimethamine, sulfadiazine, and leukovorin, while the remaining 39 were not treated. Cataracts occurred in 27 eyes of 20 patients (11.6%, 95% confidence interval [7.2%, 17.3%]). Fourteen cataracts were present at birth and 13 developed postnatally. Locations of the cataracts included anterior polar (three eyes), anterior subcapsular (six eyes), nuclear (five eyes), posterior subcapsular (seven eyes), and unknown (six eyes). Thirteen cataracts were partial, nine total, and five with unknown complexity. Twelve cataracts remained stable, 12 progressed, and progression was not known for 3. Five of 27 eyes had cataract surgery, with 2 of these developing glaucoma. Sixteen eyes of 11 patients had retinal detachment and cataract. All eyes with cataracts had additional ocular lesions. CONCLUSIONS: In the NCCCTS cohort, 11.6% of patients were diagnosed with cataracts. There was considerable variability in the presentation, morphology, and progression of the cataracts. Associated intraocular pathology was an important cause of morbidity.

Giant sarcoid tumor of the iris and ciliary body.

PURPOSE: To report the occurrence of a giant iridociliary sarcoid tumor. METHODS: The patient was evaluated by medical history, ophthalmoscopic examination (including photography and ultrasonography) as well as systemic, hematologic, and radiographic examinations. Tumor biopsies allowed for cytopathologic, histopathologic, and immunohistochemical analysis. RESULTS: The 39-year-old black male was found to have a right iris and ciliary body tumor. Ultrasonography revealed a 10 x 12-mm base, 5.6-mm height, low internal reflectivity, and vitreous debris. Radiographic imaging revealed mediastinal and bilateral hilar lymphadenopathy. A purified protein derivative (PPD) and a hematologic survey were negative. Pathology evaluations of the surgical specimens revealed features of non-caseating granulomata consistent with sarcoidosis. A combination of topical and systemic steroid therapy was locally curative. CONCLUSIONS: We describe a giant iridociliary sarcoid tumor in a patient with no lacrimal gland enlargement, conjunctival nodules, or skin lesions. A biopsy was required to establish the diagnosis.

Mechanical energy from intraocular instruments cause emulsification of silicone oil.

AIM: The complications of intraocular silicone oil include emulsification, which may lead to vision-threatening disorders such as keratopathy, secondary glaucoma and retinopathy. The authors study the effect of mechanical energy from surgical instruments on the emulsification of silicone oil. METHODS: Three different handpieces (phacofragmentation, phacoemulsification, high-speed vitrectomy) were placed at the interface of balanced salt solution and silicone oil (1000 and 5000 cst, 200 fluid; Dow Corning). The phacofragmentation handpiece was evaluated over different ultrasound powers and duration. Emulsification was quantified with phase-contrast microscopy and manual counting of digital photographs by a masked examiner. In addition, phacoemulsification was performed in the anterior chamber of a human ex vivo eye with full-fill silicone oil. RESULTS: Emulsification increases with higher phacofragmentation power and duration and is greater for 1000 cst silicone oil. Emulsification of silicone oil occurs with phacoemulsification and high-speed vitrectomy handpieces. CONCLUSIONS: The energy from surgical handpieces is sufficient to result in silicone oil emulsification.

Multifocal choroiditis in patients with familial juvenile systemic granulomatosis.

PURPOSE: To document clinical features of uveitis in patients with familial juvenile systemic granulomatosis. DESIGN: Retrospective chart review. METHODS: Ophthalmologic examination, medical history, and clinical course in 16 patients from eight families examined at six academic medical centers. RESULTS: Of the 16 patients, 15 had evidence of panuveitis with multifocal choroiditis. One patient had only an anterior uveitis. Ischemic optic neuropathy, presumably due to a small vessel vasculopathy, and retinal vasculopathy each occurred in one patient. Ocular complications were common, including cataracts in 11, glaucoma in six, band keratopathy in six, cystoid macular edema in six, and optic disk edema in six. All 16 patients had polyarthritis, and at least nine had skin rash. Often patients were misdiagnosed initially as having either juvenile rheumatoid arthritis or sarcoidosis. CONCLUSIONS: Familial juvenile systemic granulomatosis is an uncommon genetic disease characterized by polyarthritis and uveitis. Panuveitis and multifocal choroiditis often may be present. Patients with a diagnosis of juvenile rheumatoid arthritis but having a family history of the disease and multifocal choroiditis should be suspected of having familial juvenile systemic granulomatosis.