Sarju Patel

Comparison of ultra-widefield fluorescein angiography with the Heidelberg Spectralis® noncontact ultra-widefield module versus the Optos® Optomap®

Purpose To compare ultra-widefield fluorescein angiography imaging using the Optos® Optomap® and the Heidelberg Spectralis® noncontact ultra-widefield module. Methods Five patients (ten eyes) underwent ultra-widefield fluorescein angiography using the Optos® panoramic P200Tx imaging system and the noncontact ultra-widefield module in the Heidelberg Spectralis® HRA+OCT system. The images were obtained as a single, nonsteered shot centered on the macula. The area of imaged retina was outlined and quantified using Adobe® Photoshop® C5 software. The total area and area within each of four visualized quadrants was calculated and compared between the two imaging modalities. Three masked reviewers also evaluated each quadrant per eye (40 total quadrants) to determine which modality imaged the retinal vasculature most peripherally. Results Optos® imaging captured a total retinal area averaging 151,362 pixels, ranging from 116,998 to 205,833 pixels, while the area captured using the Heidelberg Spectralis® was 101,786 pixels, ranging from 73,424 to 116,319 (P = 0.0002). The average area per individual quadrant imaged by Optos® versus the Heidelberg Spectralis® superiorly was 32,373 vs 32,789 pixels, respectively (P = 0.91), inferiorly was 24,665 vs 26,117 pixels, respectively (P = 0.71), temporally was 47,948 vs 20,645 pixels, respectively (P = 0.0001), and nasally was 46,374 vs 22,234 pixels, respectively (P = 0.0001). The Heidelberg Spectralis® was able to image the superior and inferior retinal vasculature to a more distal point than was the Optos®, in nine of ten eyes (18 of 20 quadrants). The Optos® was able to image the nasal and temporal retinal vasculature to a more distal point than was the Heidelberg Spectralis®, in ten of ten eyes (20 of 20 quadrants). Conclusion The ultra-widefield fluorescein angiography obtained with the Optos® and Heidelberg Spectralis® ultra-widefield imaging systems are both excellent modalities that provide views of the peripheral retina. On a single nonsteered image, the Optos® Optomap® covered a significantly larger total retinal surface area, with greater image variability, than did the Heidelberg Spectralis® ultra-widefield module. The Optos® captured an appreciably wider view of the retina temporally and nasally, albeit with peripheral distortion, while the ultra-widefield Heidelberg Spectralis® module was able to image the superior and inferior retinal vasculature more peripherally. The clinical significance of these findings as well as the area imaged on steered montaged images remains to be determined.

Mycobacterial ocular inflammation: delay in diagnosis and other factors impacting morbidity.

IMPORTANCE The reported outcomes of ocular mycobacterial infection are commonly unfavorable. This study is among the first to elucidate factors associated with poor outcomes, as well as highlight the continued controversies in therapy, particularly the role of oral corticosteroids. OBJECTIVE To describe presentations and outcomes of mycobacterial ocular disease in the Midwestern United States. DESIGN Retrospective case series. SETTING A university-based uveitis clinic. PARTICIPANTS Twenty-six eyes of 17 patients with mycobacterial ocular inflammatory disease seen at University of Illinois at Chicago from 1995 to 2010. MAIN OUTCOME MEASURES Bivariate and regression analyses were performed to assess factors associated with delay in referral, relapse, and irreversible visual acuity loss (≤ 20/200). RESULTS Of 17 patients, 13 had isolated ocular disease, 1 had miliary tuberculosis (TB), 2 had TB lymphadenopathy, and 1 had active pulmonary TB. Fourteen had Mycobacterium tuberculosis and 3 had nontuberculous mycobacterial infection. Chest imaging was consistent with granulomatous disease in 46.7%. Average delay from ocular disease onset to uveitis service referral was 755.3 days. Posterior uveitis and non-Hispanic white race were associated with increased delay. A relapsing course was observed in posterior uveitis (odds ratio [OR], 20.0; 95% CI, 1.39-287; P = .03) and those treated with systemic steroids for eye disease (OR, 10.1; 95% CI,1.60-64.0; P = .01). Disease control was achieved in 81%, although 38.5% had profound visual loss, associated with age older than 50 years and delay in diagnosis. Patients diagnosed after 500 days from initial ocular symptoms were more likely to lose vision (OR, 20.0; 95% CI, 1.41-282; P = .03). CONCLUSIONS Ocular mycobacterial infection occurs in nonendemic areas and cannot be ruled out with negative chest imaging. Tuberculosis and atypical mycobacterial infection should be in the differential diagnosis of ocular inflammation, regardless of patient ethnicity. Significant delays exist in instituting antimicrobial treatment, associated with increased morbidity. Early referral is necessary for patients not responding appropriately to anti-inflammatory therapy.

Long-Term, Drug-Free Remission of Sympathetic Ophthalmia with High-Dose, Short-Term Chlorambucil Therapy

OBJECTIVE To evaluate the safety and effectiveness of short-term, high-dose chlorambucil therapy in achieving long-term, drug-free remission in the treatment of sympathetic ophthalmia (SO). DESIGN Retrospective case series. PARTICIPANTS Sixteen patients with SO treated with high-dose, short-term chlorambucil therapy between 1970 and 2010. METHODS Descriptive and bivariate analyses were used to characterize disease and outcomes. MAIN OUTCOME MEASURES Months of disease-free remission, prevalence rate of relapse, and prevalence of serious treatment-related adverse events. RESULTS Sixteen patients with SO treated with short-term, high-dose chlorambucil were identified. Patients were treated with chlorambucil for a median of 14.0 weeks (mean, 14.5 weeks; range, 12.0-19.0 weeks). Median follow-up was 98.5 months (mean, 139.1 months; range, 48-441 months) from initiation of chlorambucil therapy. Control of inflammation was achieved in 100% of patients. Thirteen patients (81.3%) maintained vision of 20/40 or better in the sympathizing eye. Four patients (25%) relapsed after a median of 83 months (mean, 131 months) after cessation of systemic therapy. Seventy-five percent of relapses were controlled with topical therapy only. Conjunctival Kaposis sarcoma developed in 1 patient. No patient demonstrated systemic malignancy. CONCLUSIONS Short-term, high-dose chlorambucil therapy provides sustained periods of drug-free remission. With median follow-up of more than 8 years (mean, 11.6 years; range, 4-37 years), there was a low rate of recurrence and minimal long-term serious health consequences or adverse events. Because SO may be a lifelong condition and because chlorambucil therapy may offer long-term, drug-free remission, this treatment may be worth considering early in the decision-making process for severe sight-threatening disease.

Immunologic and genetic markers in patients with idiopathic ocular inflammation and a family history of inflammatory bowel disease

PURPOSE To evaluate the prevalence of immunologic and genetic markers in patients with idiopathic ocular inflammation and a family history of inflammatory bowel disease. DESIGN Matched case-control study. METHODS Patients with a diagnosis of idiopathic ocular inflammation and family history of inflammatory bowel disease who did not have inflammatory bowel disease themselves were identified and matched to control patients with idiopathic ocular inflammation. Serum was evaluated for immunologic markers using Prometheus IBD Serology 7. Genomic DNA was analyzed for single nucleotide polymorphisms (SNP) of the NOD2 gene associated with Crohn disease. RESULTS Fifteen patients with idiopathic ocular inflammation and family history of inflammatory bowel disease were matched to 15 control patients based on age, sex, and race. Eight of 15 patients (53%) with a family history of inflammatory bowel disease had elevated p-ANCA antibody levels compared to 3 of 15 controls (20%) (1-sided P = .04) with a matched analysis odds ratio of 6.0 (1-sided P = .06). Four of 15 patients (27%) with family history of inflammatory bowel disease tested positive for immunologic markers predicting ulcerative colitis, while no control patients tested positive (1-sided P = .06). Carrier rates of NOD2 SNPs did not differ significantly between the test and control groups. CONCLUSIONS One-quarter of patients with idiopathic ocular inflammation and a family history of inflammatory bowel disease had immunologic markers predicting bowel disease, and one-half had elevated p-ANCA levels. Prometheus IBD Serology 7 may be useful in the evaluation of selected patients with unexplained uveitis.

Progressive outer retinal necrosis in a multiple sclerosis patient on natalizumab

A 54-year-old woman with multiple sclerosis, on natalizumab, reported 2 weeks of left eye redness and blurry vision, previously diagnosed as conjunctivitis. Visual acuity was 20/125. Funduscopy revealed progressive outer retinal necrosis (figure 1), a viral retinitis of immunocompromised patients that frequently causes vision loss.1 Sequential bilateral involvement is common. Etiologies include varicella zoster, herpes simplex, and rarely cytomegalovirus; aqueous PCR revealed varicella. Natalizumab was held. IV and intravitreal antivirals were administered. After repair of retinitis-induced retinal detachment and resolution of retinitis, visual acuity was 20/125 (figure 2). In immunocompromised patients with ocular symptoms, clinicians should consider infectious retinitis and obtain prompt funduscopic evaluation.

Directed conjunctival biopsy and impact of histologic sectioning methodology on the diagnosis of ocular sarcoidosis

BackgroundSarcoidosis is an idiopathic, multi-system, granulomatous disease with well-described ocular manifestations. However, other uveitic etiologies can manifest in a similar fashion, and ocular disease may precede systemic manifestations. Definitive diagnosis requires histologic confirmation of non-caseating granulomatous inflammation. This study reports the diagnostic yield of directed biopsy of conjunctival follicles in patients with uveitis suspected to be secondary to sarcoidosis, and compares an institutional standard tissue sectioning method to a multi-plane technique.ResultsA retrospective analysis was performed of all patients who underwent directed conjunctival biopsy for suspected ocular sarcoidosis. A total of eight patients were identified; all were females. Directed conjunctival biopsy was positive in three of seven patients using standard histologic processing method, a yield of 43%. Using the multi-plane technique increased the cumulative yield to 63%.ConclusionsDirected conjunctival biopsy is a minimally invasive, cost-effective, and moderately high yield method of diagnosing ocular sarcoidosis. Using a multi-plane sectioning method may increase biopsy yield when standard sectioning techniques are negative.

SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS IN MACULA-INVOLVING CYTOMEGALOVIRUS RETINITIS

Purpose: To evaluate the microstructural features of cytomegalovirus (CMV) retinitis by spectral domain optical coherence tomography (OCT). Methods: Subjects were patients with macula-involving CMV retinitis with OCT imaging. The leading edge of retinitis in the macula was identified based on fundus imaging, and OCT findings were longitudinally evaluated in three areas: within the area of active retinitis, at the leading edge of retinitis, and just beyond the leading edge of retinitis. Results: Optical coherence tomography imaging of macular CMV retinitis identified vitreous cells in 10 eyes (100%), posterior vitreous detachment in four eyes (40%), broad-based vitreomacular traction in one eye (10%), epiretinal membrane in eight eyes (80%), and lamellar hole–associated epiretinal proliferation associated with an atrophic hole in one eye (10%). Retinal architectural disruption, disruption of inner retinal layers, disruption of the external limiting membrane, and ellipsoid zone abnormalities were noted within the area of retinitis in all eyes and decreased in frequency and severity at and beyond the leading edge of retinitis, although all 10 eyes (100%) exhibited one of these abnormalities, especially outer retinal microabnormalities, beyond the leading edge of retinitis. Conclusion: Microstructural abnormalities were frequently noted on OCT of CMV retinitis, including within the retina beyond the leading edge of retinitis identified by corresponding fundus imaging. Outer retinal abnormalities were noted more frequently than inner retinal abnormalities beyond the leading edge of retinitis. These findings provide insight into the effects of CMV retinitis on retinal microstructure and potentially on vision and highlight the potential utility of OCT for monitoring microprogression of macula-involving CMV retinitis.

Infection of the optic apparatus and hypothalamus by Mycobacterium haemophilum

Mycobacterium haemophilum is a nontuberculous mycobacterium that primarily manifests as cutaneous ulcerations in immunocompromised adults and cervical lymphadenitis in immunocompetent children.1 We present a case of M haemophilum infection of the optic chiasm, tract, and hypothalamus in a patient with AIDS.

Acanthamoeba endophthalmitis during treatment for cutaneous disease in a renal transplant patient

Acanthamoeba infections are difficult to diagnose and treat. We present a renal transplant patient who developed Acanthamoeba endophthalmitis on therapy with posaconazole and miltefosine for cutaneous acanthamobiasis. The patient was maintained on intracameral voriconazole injections, and oral azithromycin, fluconazole, and flucytosine. This case highlights novel presentations and treatments for acanthamoebic infection.

Relapse of Juvenile Idiopathic Arthritis-Associated Uveitis after Discontinuation of Immunomodulatory Therapy

ABSTRACT Purpose: To assess treatment outcomes in juvenile idiopathic arthritis (JIA)-associated uveitis and relapse rates upon discontinuation of immunomodulatory therapy (IMT). Methods: Medical records of patients with JIA-associated uveitis seen at the University of Illinois at Chicago and the F.I. Proctor Foundation uveitis clinics from September 14, 1988 to January 5, 2011 were reviewed. The main outcome was time to relapse after attempting to discontinue IMT.Results: Of 66 patients with JIA-associated uveitis, 51 (77%) received IMT as either sole or combination therapy. Of a total of 51, 41 (80%) patients achieved corticosteroid-sparing control. Attempts were made to discontinue treatment in 19/51 (37%) patients. Of a total of 19 patients, 13 (68%) attempting to discontinue IMT relapsed, with a median time to relapse of 288 days from the time of attempted taper/discontinuation (IQR: 108–338).Conclusions: Corticosteroid-sparing control of inflammation was achieved in the majority of patients; however, attempts to stop IMT were often unsuccessful. Close follow-up of patients after discontinuation of therapy is warranted.