Akshay S. Thomas

Ocular manifestations of inflammatory bowel disease.

Purpose of review Extraintestinal manifestations (EIMs) of inflammatory bowel disease (IBD) are numerous and can often involve the eye. This review highlights the ocular complications associated with IBD including the critical role the ophthalmologist can play in the diagnosis of IBD, the pathogenesis of IBD, its ocular complications, and the treatment of ocular inflammation associated with IBD. Recent findings Polygenic and environmental influences, as well as gut microbial dysbiosis, have been implicated in the pathogenesis of IBD. IBD and its EIMs appear to respond well to TNF&agr;-targeted biologics. Summary IBD is thought to be caused by polygenic and environmental influences, including a dysbiotic gut microbiota. It is a systemic immune-mediated disease with varying types of ocular manifestations that can precede, occur simultaneously, or follow intestinal involvement. The diagnosis of IBD can be confused with other seronegative spondyloarthropathies as well as Behçets disease. Treatment of IBD-associated ocular inflammation can range from corticosteroids to steroid-sparing immunosuppression such as azathioprine or methotrexate. Refractory disease can respond well to TNF&agr; inhibitors.

Effect Of Systemic Beta-blockers, Ace Inhibitors, And Angiotensin Receptor Blockers On Development Of Choroidal Neovascularization In Patients With Age-related Macular Degeneration

Purpose: Recent studies have suggested that the use of systemic beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers can induce regression of choroidal neovascularization in rodent models. The purpose of this study is to evaluate if these agents have a protective effect against the development of choroidal neovascularization in patients with age-related macular degeneration. Methods: In this single-center retrospective case–control study, the charts of 250 patients with neovascular age-related macular degeneration were compared with those of 250 controls with dry age-related macular degeneration. Charts were reviewed for current and past use of beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers. Frequency tables were generated, and associations were examined using chi-square tests, t-tests, and multivariate logistic regression. Results: There was no statistically significant difference between rates of beta-blocker use (P = 0.57), angiotensin-converting enzyme inhibitors use (P = 0.20), or angiotensin receptor blockers use (P = 0.61) between the 2 groups. Additionally, there was no statistically significant difference between rates of use of combinations of the above drugs between the two groups. Conclusion: Although there is growing evidence that beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers can induce regression of choroidal neovascularization in rodent models, these medications do not seem to confer a protective effect against the development of choroidal neovascularization in patients with age-related macular degeneration.

Spectral-Domain Optical Coherence Tomography and Fundus Autofluorescence Evaluation of Torpedo Maculopathy.

The authors describe the spectral-domain optical coherence tomography and fundus autofluorescence findings in a case of torpedo maculopathy. Spectral-domain optical coherence tomography revealed loss or disruption of the retinal pigment epithelium and overlying disruption of the outer neurosensory retina. Fundus autofluorescence revealed reduced fundus autofluorescence of the lesion surrounded by a rim of increased fundus autofluorescence.The authors describe the spectral-domain optical coherence tomography and fundus autofluorescence findings in a case of torpedo maculopathy. Spectral-domain optical coherence tomography revealed loss or disruption of the retinal pigment epithelium and overlying disruption of the outer neurosensory retina. Fundus autofluorescence revealed reduced fundus autofluorescence of the lesion surrounded by a rim of increased fundus autofluorescence.

The Impact and Implication of Peripheral Vascular Leakage on Ultra-Widefield Fluorescein Angiography in Uveitis

ABSTRACT Purpose: To study if peripheral vascular leakage (PVL) on ultra-widefield fluorescein angiography (UWFFA) prognosticates complications of uveitis or necessitates treatment augmentation. Methods: Retrospective cohort study of uveitis patients imaged with UWFFA and ≥1 yr of follow-up. Results: We included 73 eyes of 42 patients with uveitis. There was no difference in baseline, intermediate, final visual acuity (p = 0.47–0.95) or rates of cystoid macular edema (CME) (p = 0.37–0.87) in eyes with PVL vs. those without. Eyes with PVL receiving baseline treatment augmentation were more likely to have baseline CME but were not more likely to have impaired visual acuity at final follow-up. PVL was independently associated with treatment augmentation on generalized estimating equation analysis with multivariable linear regression (OR: 4.39, p = 0.015). Conclusions: PVL did not confer an increased risk of impaired VA or CME at ≥1 yr follow-up but was possibly an independent driver of treatment augmentation.

Macular Holes, Vitelliform Lesions, And Midperipheral Retinoschisis In Alport Syndrome

PURPOSE To describe the retinal findings in two cases of Alport syndrome. METHODS Observational case series. The clinical findings of the two patients were documented with color fundus photography and high resolution spectral domain optical coherence tomography. RESULTS Patient 1 was found to have fleck retinopathy in both eyes, inner retinal thinning in the right eye and a full-thickness macular hole in the left eye. Patient 2 was found to have a full-thickness macular hole in the right eye as well as retinoschisis in the temporal macula in the right eye. The left eye revealed inner retinal thinning involving the fovea, a vitelliform lesion of the temporal macula and midperipheral retinoschisis involving multiple retinal layers. CONCLUSION Retinal abnormalities including fleck retinopathy, retinal thinning, macular holes, retinoschisis, and vitelliform lesions are variably present in Alport syndrome. This is only the second report of a vitelliform lesion in a patient with Alport syndrome and the first report of midperipheral retinoschisis. The array of retinal findings is believed to reflect a dysfunctional Type IV collagen present in the internal limiting membrane and Bruch membrane.

Long-Term Surgical Outcomes of Retinal Detachment Associated With Acute Retinal Necrosis

BACKGROUND AND OBJECTIVE To determine the long-term visual and surgical outcomes of patients with acute retinal necrosis (ARN) associated retinal detachment (RD). PATIENTS AND METHODS The authors conducted a single-center, retrospective chart review from 2001 to 2012 of 32 eyes from 27 patients diagnosed with ARN. The authors assessed the rates and risk factors for recurrent RD in eyes having undergone primary RD repair for ARN-related RD. RESULTS Fifteen eyes (46.9%) developed RD and 13 underwent surgical repair. Recurrent RD developed in six eyes (46.2%), occurring 35 days to 10 months after primary retinal surgery. There was no difference in the rate of recurrent RD between eyes treated with or without intravitreal foscarnet (P = .48) or initial scleral buckle (P = .31). Six eyes (46.2%) developed severe vision loss, with a final Snellen visual acuity of less than 20/200. CONCLUSION Recurrent RD after primary repair is a frequent complication of ARN. Overall, visual prognosis is guarded despite surgical intervention. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:660-664.].

Vitreoretinal surgery in uveitis.

The underlying cause of uveitis is not always clear. Surgery may be helpful in some cases to help determine a diagnosis and potentially guide therapeutics and management. Diagnosis is particularly important in masquerade syndromes and infectious uveitis where targeted therapy can dramatically improve visual outcomes. Although certain entities (eg, syphilitic uveitis, lyme disease–related uveitis) can be diagnosed based on serology, others such as toxoplasma chorioretinitis and herpetic eye disease cannot owing to seropositivity in a large proportion of unaffected individuals. In general, surgical collection of aqueous, vitreous, or chorioretinal samples are indicated when (1) there is concern for an infectious etiology, (2) there is concern for malignancy, or (3) when empiric medical management has failed to control the intraocular inflammation. Several studies have reported the benefit of vitrectomy on the clinical course of chronic uveitis with improvement in visual acuity, decreased cystoid macular edema (CME), decreased incidence of uveitic exacerbations, and reduction in the amount of immunosuppressive medications required. The main indications for a therapeutic vitrectomy in uveitis patients include: (1) recalcitrant CME, (2) media opacities involving the visual axis, (3) retinal detachment, (4) cyclitic membrane causing hypotony, (5) epiretinal membrane (ERM), (6) control of inflammation, (7) endophthalmitis. In all cases of elective vitreoretinal surgery, it is ideal to have inflammation under control for 3 months before surgery. Below we discuss the evidence supporting vitreoretinal surgery in the diagnosis and management of uveitis and its complications.

Association of Disorganization of Retinal Inner Layers with Ischemic Index and Visual Acuity in Central Retinal Vein Occlusion

Purpose To determine whether disorganization of retinal inner layers (DRIL) on optical coherence tomography (OCT) is associated with ischemia on ultra-widefield fluorescein angiography (UWFFA) and with visual outcomes in eyes with acute, treatment-naïve central retinal vein occlusion (CRVO). Design Retrospective, single-institution, longitudinal cohort study. Participants Twenty-five consecutive patients with treatment-naïve CRVO and ≥ 1 year follow-up. Methods Two independent masked graders evaluated the extent of DRIL, ellipsoid zone disruption, external limiting membrane disruption, and other OCT parameters at the baseline, 6- month, 12-month, and final visits. Baseline UWFFA images were assessed for ischemic index values and foveal avascular zone (FAZ) enlargement. Main Outcome Measures Associations of DRIL with UWFFA findings and clinical outcomes including corrected visual acuity (VA). Results The median time to final follow-up was 24 months (range 12.1 - 43.9 months). Median DRIL extent at baseline was 765 µm (range 0 - 1000 µm). Eighteen of 25 eyes (72%) had some degree of DRIL at baseline, and 20 of 25 eyes (80%) had cystoid macular edema (CME). Neither the presence nor extent of DRIL at baseline was associated with presenting VA. In a cross-sectional analysis of each visit, extent of DRIL correlated with worse VA at both the 6-month (ρ = 0.656; p = 0.001) and final (ρ = 0.509; p = 0.016) visits. At final follow-up, DRIL extent was the OCT parameter most strongly correlated with baseline ischemic index (ρ = 0.418; p = 0.047) and baseline enlarged FAZ (p = 0.057) on UWFFA. On multivariate regression analysis, DRIL extent at final follow-up was the only OCT parameter associated with worse VA (p = 0.013) and remained significant when accounting for CME as a potential confounder. Conclusions Extent of DRIL was not associated with presenting VA in treatment-naïve eyes with acute CRVO. Following six months of follow-up however, DRIL extent correlated with worse VA and was predictive of worse VA throughout more than 2 years of follow-up. Ischemic features on UWFFA at baseline are predictive of the extent of DRIL development at final follow-up.

Longitudinal Follow-Up of Choroidal Thickness in Central Retinal Vein Occlusion With and Without Cystoid Macular Edema

Purpose: To investigate the longitudinal relationship between subfoveal choroidal thickness (CT) and central retinal vein occlusion (CRVO). Methods: Retrospective cohort of 104 subjects with enhanced-depth imaging optical coherence tomography for unilateral CRVO. Mean CT and best-corrected visual acuity (BCVA) were compared in eyes with and without CRVO and in eyes with CRVO with and without cystoid macular edema (CME). Results: CT was thicker in eyes with CRVO-related CME than uninvolved contralateral eyes at baseline (263.9 ± 86.9 versus 230.2 ± 87.9 µm; P < .001) and final follow-up (261.1 ± 94.7 versus 222.3 ± 86.2 µm; P = .007). CRVO eyes treated with intravitreal antivascular endothelial growth factor with or without steroid therapy showed a significant reduction in CT at final follow-up (256.3 ± 90.7 versus 236.9 ± 85.9 µm; P = .004). Subjects with CRVO who were not treated with intravitreal injections also showed a significant but more modest decline in CT over time (234.4 ± 94.2 versus 221.5 ± 97.1 µm; N = 31; P = .02). However, contralateral uninvolved eyes without CRVO did not show a significant change in CT over time (233.3 ± 87.9 versus 219.5 ± 90.6 µm; N = 71; P = .40). Persistent CME at final follow-up was associated with thicker baseline (277.6 ± 96.4 versus 235.1 ± 86.5 µm; P = .02) and final CT (265.7 ± 93.4 versus 215.0 ± 82.1 µm; P = .005). Change in CT was not related to change in BCVA (P > .05). Conclusions: CT was greater in eyes with CRVO-related CME compared to eyes with CRVO but no CME and compared to uninvolved contralateral eyes. CT decreased in eyes with CRVO over time both among eyes that received intravitreal injections and among eyes that did not receive injections. CT may be a prognosticator of treatment response in CRVO-related CME.

Orbital arteriovenous fistula presenting with choroidal pulsations on optical coherence tomography

Purpose To describe a case of orbital arteriovenous fistula diagnosed based on choroidal pulsations on optical coherence tomography (OCT). Observations A 69-year-old female originally referred for evaluation of macular degeneration. During acquisition of OCT images, choroidal pulsations of the right eye were noted on the B-scan on the instrument display. The pulsations were not noted on gross or funduscopic examination. Fluorescein angiography was unremarkable. Indocyanine green angiography revealed engorged choroidal vasculature in the right eye. OCT angiography revealed relative tortuosity and dilation of the superficial and deep vascular complexes respectively. B-scan ultrasonography revealed orbital pulsations on the right. MRI imaging of the brain and orbits was unremarkable. Findings were attributed to a low-flow orbital arteriovenous fistula. The patient subsequently developed mild stasis retinopathy for which anti-VEGF therapy was initiated. Conclusions and importance Arteriovenous fistulas of the brain and orbit classically present with gross pulsatile proptosis among other clinical features. Low flow orbital fistulas may present with subtle choroidal pulsations only detectable on OCT.