Manjot K. Gill

Variations in the Presentation of Primary Intraocular Lymphoma: Case Reports and a Review

Primary intraocular lymphoma is a distinct subset of primary non-Hodgkins lymphoma of the central nervous system (CNS). Diagnosis can be difficult and is often delayed, as the clinical presentation can mimic a number of other ocular conditions. This report describes four different presentations of intraocular lymphoma and focuses on its modes of clinical presentation. Primary intraocular lymphoma can present with a wide variety of manifestations frequently mimicking diffuse uveitis that is refractory to corticosteroids. Subretinal pigment epithelium tumors may be seen. However, other presentations may include multiple deep white dots in the retina secondary to tumor infiltration; retinal infiltration, causing a necrotizing retinitis; or infiltration of the retinal vasculature, causing arterial or venous obstruction. Finally, optic nerve invasion may be seen. CNS lymphoma develops in the majority of patients before, in conjunction with, or after the development of eye disease. Intraocular lymphoma often has a fatal outcome, but recognition of its modes of presentation facilitates early diagnosis and treatment that may improve prognosis.

Visual and anatomical outcomes following intravitreal aflibercept in eyes with recalcitrant neovascular age-related macular degeneration: 12-month results

PurposeTo describe the efficacy of intravitreal aflibercept on 12-month visual and anatomical outcomes in patients with neovascular age-related macular degeneration (AMD) recalcitrant to prior monthly intravitreal bevacizumab or ranibizumab.MethodsNon-comparative case series of 21 eyes of 21 AMD patients with evidence of persistent exudation (intraretinal fluid/cysts, or subretinal fluid (SRF), or both) on spectral domain OCT despite ≥6 prior intravitreal 0.5 mg ranibizumab or 1.25 mg bevacizumab (mean 29.8±17.1 injections) over 31.6±17.4 months who were transitioned to aflibercept.ResultsAt baseline, best-corrected visual acuity (BCVA) was 0.42±0.28 logarithm of minimum-angle of resolution (logMAR), central foveal thickness (CFT) was 329.38±102.67 μm and macular volume (MV) was 7.71±1.32 mm3. After 12 months of aflibercept (mean 10.2±1.2 injections), BCVA was 0.40±0.28 logMAR (P=0.5), CFT decreased to 292.71±91.35 μm (P=0.038) and MV improved to 7.33±1.27 mm3 (P=0.003). In a subset of 15 eyes with a persistent fibrovascular or serous pigment epithelial detachment (PED), mean baseline PED greatest basal diameter (GBD) was 2350.9±1067.6 μm and mean maximal height (MH) was 288.7±175.9 μm. At 12 months, GBD improved to 1896.3±782.3 μm (P=0.028), while MH decreased to 248.27±146.2 μm (P=0.002).ConclusionIn patients with recalcitrant AMD, aflibercept led to anatomic improvement at 12 months, reduction in proportion of eyes with SRF and reduction in PED, while preserving visual acuity.

Quantification of Change in Pigment Epithelial Detachment Volume and Morphology After Transition to Intravitreal Aflibercept in Eyes With Recalcitrant Neovascular AMD: 18-Month Results

BACKGROUND AND OBJECTIVE To quantitatively evaluate the change in pigment epithelial detachment (PED) morphology on spectral-domain optical coherence tomography (SD-OCT) 18 months after the transition to intravitreal aflibercept in patients with neovascular age-related macular degeneration (AMD) with PED recalcitrant to monthly intravitreal bevacizumab or ranibizumab. PATIENTS AND METHODS Retrospective case series examining patients with neovascular AMD who had a persistent fibrovascular or serous PED on SD-OCT. PED volume was calculated by manually outlining the PED on individual OCT slices of the raster scan and multiplying by the pixel dimensions. RESULTS Eleven eyes of 10 patients who had received an average of 25.7 ± 20.1 (range: 6 to 70) prior bevacizumab or ranibizumab injections over a period of 26.6 ± 19.8 months (range: 4 to 63) were included. PED volume decreased with aflibercept from 0.687 ± 0.837 mm(3) to 0.562 ± 0.705 mm(3) (P = .02), a decrease of 19% ± 12.27%. CONCLUSION After 18 months of aflibercept, recalcitrant PED volumes were reduced by 19% while preserving visual acuity in eyes with neovascular AMD.

Transitioning to intravitreal aflibercept following a previous treat-and-extend dosing regimen in neovascular age-related macular degeneration: 24-month results

Purpose:To evaluate frequency of injections, visual and anatomical outcomes of neovascular age-related macular degeneration (nAMD) patients transitioned to intravitreal aflibercept after failure to extend treatment interval beyond 8 weeks with prior intravitreal bevacizumab or ranibizumab.Methods:Retrospective review of patients with nAMD switched to aflibercept following ≥6 prior intravitreal ranibizumab or bevacizumab injections at 4–8-week intervals. Three monthly aflibercept injections were given followed by a treat-and-extend dosing regimen.Results:Twenty-one eyes of 18 patients who had received a mean of 23.8±18.8 (mean±SD; range 6–62) prior ranibizumab or bevacizumab injections were included. Over a mean follow-up of 24 months after the transition, 9.2±2.9 (range 4–21) aflibercept injections were required. Interval between aflibercept injections increased to 57.3 days (range 35–133 days), as compared with 37±6.1 days (range 29–54 days) with the prior agents (P=0.01). Mean best-corrected visual acuity was preserved (0.42±0.31 vs 0.42±0.23 logMAR; P=0.2). Mean OCT central subfoveal thickness (292.1±83.2 μm to 283.6±78.6 μm; P=0.4) and mean macular volume (7.9±0.95 mm3 to 7.67±0.94 mm3; P=0.16) remained stable.Conclusion:Patients requiring treatment more frequently than every 8 weeks with ranibizumab and bevacizumab were transitioned to >8-week treatment interval with aflibercept while maintaining the anatomic and visual gains.

Spectral Domain Optical Coherence Tomography In The Evaluation And Management Of Infectious Retinitis

Purpose: To describe spectral domain optical coherence tomography (SD-OCT) findings of infectious retinitis, including affected layer of retinal involvement, changes at the vitreoretinal interface, and response to therapy. Methods: Observational case series. A retrospective review of five patients with infectious retinitis: one with toxoplasmosis, three with herpetic retinitis secondary to cytomegalovirus, and one with herpetic retinitis secondary to varicella zoster virus. Each patient underwent a complete ophthalmologic examination, fundus photography, and SD-OCT imaging (Heidelberg Spectralis; Heidelberg Engineering, Heidelberg, Germany) of the affected retina at the initial visit with serial fundus photography and SD-OCT imaging at follow-up visits. Approval was obtained from the Institutional Review Board of Northwestern University. Results: Spectral domain ocular coherence tomography of retinitis associated with Toxoplasma, cytomegalovirus, or varicella zoster virus demonstrates full-thickness disruption of the retinal architecture and overall thickening. This was in contrast to clinically imitating lesions such as cotton-wool spots, which only showed focal swelling of the inner retina. There was a clear demarcation between the area of active retinitis and unaffected retina. Inactivity was apparent when the previously affected thickened area became atrophic. The SD-OCT also demonstrated changes at the vitreoretinal interface where there was frequently a detachment of the posterior hyaloid (four of five cases) associated with overlying vitreous debris and formation of tractional changes. In the case of varicella zoster virus retinitis, this traction subsequently led to a total retinal detachment. Conclusion: In the assessment of infectious retinitides, SD-OCT is a helpful adjunct to clinical examination and fundus photography. It provides high-resolution detail regarding the border of infectious activity, the vitreoretinal interface, and the differentiation of lesions that can clinically mimic active retinitis. Serial SD-OCT also provides further insight into response to therapy and postinfectious retinal changes by highlighting areas that are at greater risk for complications such as retinal detachment.

Traumatic macular holes.

A macular hole is a full-thickness defect of the retinal tissue involving the anatomical fovea of the eye. The first macular hole descriptions were made in the second half of the nineteenth century. Early case descriptions of macular holes focused on young, traumatized eyes. Knapp made the first case description of a macular hole in 1869 in a patient with ocular contusion injury and an initial diagnosis of macular hemorrhage. He and most other observers attributed macular holes to ocular trauma. Two years later, Noyes made the first accurate and detailed ophthalmoscopic description of a macular hole that was secondary to blunt trauma in a 13year-old girl. Noyes was the first to recognize that the hallmark of the lesion was a full-thickness defect within the center of the macula. He postulated that blunt trauma could cause immediate macular hole formation from mechanical energy created by vitreous fluid waves and contrecoups macular necrosis or macular laceration. The first histopathological descriptions of full-thickness holes were provided by Fuchs (1901) and by Coats. Coats noted cystic intraretinal changes adjacent to the macular holes and thought that these changes could be caused by ocular trauma and by other atraumatic mechanisms. The coalescence of these intraretinal cysts could then lead to a full-thickness macular hole. Even in the early part of the twentieth century, traumatic holes were believed to account for one-half of all cases of macular holes. Beginning with Schepens in 1955, the role of the vitreous in macular hole formation was postulated and, with Gass, a comprehensive description of the stages of macular hole development and the role of the prefoveolar vitreous cortex in macular hole formation was delineated. Thus, although these early case reports of macular holes described traumatic macular holes, it is now known that

Management of eyes with both idiopathic macular hole and choroidal neovascularization.

Purpose To describe the characteristics, treatment, and outcome of five eyes with both choroidal neovascularization (CNV) and macular hole. Methods Medical records of five patients with both macular hole and CNV were reviewed. Results All eyes had full-thickness macular holes. Most eyes had atypical-appearing macular holes (subretinal hemorrhage, prominent subretinal fluid, or discoloration at the hole margin) at presentation or subsequently when CNV developed. Fluorescein angiography (FA) confirmed the presence of CNV in each eye. Three eyes underwent combined macular hole repair and CNV removal, and sustained closure of these macular holes was achieved. A fourth eye underwent successful argon laser photocoagulation of extrafoveal CNV, and macular hole surgery was declined. The final eye underwent two macular hole repairs before sustained closure was achieved. Final visual acuity, ranging from 20/100 to hand motions, was limited by macular pathology and/or cataract. Conclusions Choroidal neovascularization can occur in association with a macular hole. In eyes with an atypical-appearing macular hole, FA should be obtained to detect CNV. Excision of the CNV can be done safely in conjunction with macular hole surgery. Final visual acuity may be limited by cumulative retinal and retinal pigment epithelium damage, especially in eyes with underlying macular disease.

Melanocytoma of the Optic Disc Associated With Visual Field Defects: Clinical Features and Imaging Characteristics

The authors studied the clinical features, visual field, and spectral-domain optical coherence tomography (SD-OCT) characteristics of peripapillary melanocytoma associated with visual field defects in two eyes of two patients. Both eyes had hyperreflective anterior surface and posterior optical shadowing in the region of peripapillary melanocytoma on SD-OCT imaging. The retinal nerve fiber layer was thin adjacent to these lesions, and this corresponded to the opposite hemifield defect on Humphrey Visual Field testing. Three-dimensional imaging of the melanocytoma revealed an irregular elevation in the peripapillary region. SD-OCT, when used in conjunction with clinical examination and Humphrey Visual Field testing, provided useful information that may assist clinicians in treating patients with peripapillary melanocytoma, particularly when observing them over time.

SEMIAUTOMATED QUANTITATIVE APPROACH to CHARACTERIZE TREATMENT RESPONSE in NEOVASCULAR AGE-RELATED MACULAR DEGENERATION: A Real-World Study

Purpose: To perform a quantitative study of the vascular microstructure in actively treated choroidal neovascularization by optical coherence tomographic angiography. Methods: Patients undergoing individualized anti–vascular endothelial growth factor therapy of minimum 12 months duration were included in this cross-sectional observational study and imaged using optical coherence tomographic angiography. En face optical coherence tomographic angiography images were analyzed for quantitative features, such as junction density, vessel length, and lacunarity using validated software (Angiotool). Patients were divided into 2 groups depending on their individualized treatment interval: “good responders, treated less frequently than 6 weeks” versus “poor responders, treated every 6 weeks or more frequently.” Nonparametric testing was used to assess differences between these groups. Results: Twenty-five eyes of 23 consecutive patients with a median 58-month history of choroidal neovascularization, treated by median of 34 anti–vascular endothelial growth factor injections, were included in the analysis. There was no significant difference between any of the microvascular choroidal neovascularization features between the 2 groups (P > 0.05). Conclusion: The semiautomated vessel segmentation software provides an objective and quantitative approach for choroidal neovascularization characterization. The consistently nonsignificant outcomes between the groups may provide evidence to support the “normalization hypothesis.” This would suggest that regardless of treatment interval, individualized therapy in these eyes established vessel stability.

New insights into acute annular outer retinopathy

PURPOSE Acute annular outer retinopathy is a rare entity. There are only a few cases in the worlds literature. Our study is the first to describe the spectral domain optical coherence tomography (OCT) findings in the acute presentation of this disease and to follow the disease in convalescence. METHODS This article is a descriptive case report. We used Spectralis OCT to image the transient intraretinal whitening that is initially seen in this disorder, and the OCT changes during follow-up. RESULTS Our article provides additional information about the natural history of this uncommon disease, and its varied presentation and prognosis. We were able to document the initial white line seen in the acute phase of the disease and correlate it anatomically by using OCT. We found hyperreflectivity in the outer nuclear layer and the Henle fiber layer along with marked atrophy of the outer retina within the white ring. We documented restoration of foveal photoreceptors, which corresponded with visual recovery. We also noted progressive atrophy in areas where the white ring was previously visible. CONCLUSION We describe a single case of acute annular outer retinopathy with the corresponding spectral domain OCT findings during the acute phase of the disease and during subsequent follow-up demonstrating progressive atrophy of the outer retina. This may shed insight into possible causation for this rare disease.