Orbital marginal cell lymphoma and mantle cell lymphoma subclone in patient with monoclonal gammopathy of unknown significance.

Indolent lymphomas have the potential to transform into more aggressive phenotypes. This phenomenon is best exemplified by the transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma into diffuse large B-cell lymphoma. However, it is less common to find multiple small cell lymphomas in the orbit, particularly mantle cell lymphoma. We present a case of an extranodal marginal zone lymphoma arising in the orbit that acquired a subclonal t(11;14) (q13;32) CCND1-IGH translocation, giving rise to a subclone of mantle cell lymphoma. The management of this tumor was targeted towards the more aggressive mantle cell subclone. The tumor exhibited an incomplete response to rituximab alone. To the best of the authors' knowledge, this represents the first such case to be described irrespective of site of origin.

Cutaneous T-cell lymphoma of the eyelid masquerading as dermatitis.

Cutaneous T-cell lymphoma (CTCL) may pose a diagnostic challenge for physicians, as clinical presentation and histologic analysis may mimic benign dermatologic conditions. The authors present a case of recurrent CTCL in which the recurrence was limited to the eyelid and misdiagnosed as a contact dermatitis. To the best of the authors' knowledge, this is the first reported case of recurrent CTCL that has presented solely as dermatitis of the eyelid.

Clinical Evaluation of Patients with Retinitis Pigmentosa.

This chapter describes the clinical evaluation process of patients with retinitis pigmentosa (RP). The clinical evaluation consists of a complete history and ophthalmic examination. Here, we outline the aspects of the history and ophthalmic exam that are most important for the evaluation of RP patients. In addition, the expected findings of RP patients and the etiology of these findings are discussed.

Measure of Visual Function.

This chapter describes various methods of the assessment of visual function used for assessing disease progression and treatment response in patients with retinitis pigmentosa (RP). These methods include full-field stimulus testing (FST), near-infrared autofluorescence (NIR-AF), quantitative fundus autofluorescence (qAF), and quantitative near-infrared autofluorescence (qNIR-AF). This chapter will also outline the protocol for adaptive optics (AO) imaging of RP patients and cover how each of these methods is used for RP patients, with details including the expected findings, as evidenced by recent literature.

Remote Contrast Sensitivity Testing Seems to Correlate With the Degree of Glaucomatous Macular Damage.

PRCIS: Remote contrast sensitivity (CS) testing through a free downloadable home test correlates with glaucomatous macular damage measured by 10-2 visual field (VF) testing. PURPOSE: To assess the feasibility and validity of home CS monitoring as a measure of glaucomatous damage using a free downloadable smartphone application. METHODS: Twenty-six participants were asked to remotely use the Berkeley Contrast Squares (BCS) application, a free downloadable tool that records the user's CS for varying degrees of visual acuity. An instructional video detailing how to download and use the application was sent to the participants. Subjects were asked to send logarithmic CS results with a minimum 8-week test-retest window, and test-retest reliability was measured. Results were validated against office-based CS testing that was collected within the previous 6 months. Validity analysis was also carried out to determine whether CS as measured by BCS is a good predictor of 10-2 and 24-2 VF mean deviation (MD). RESULTS: There was a high BCS test-retest reliability with an intraclass correlation coefficient score of 0.91 and a significant correlation between repeat test results and baseline test scores (Pearson, 0.86, P < 0.0001). There was significant agreement between unilateral CS scores as measured by BCS and office-based CS testing ( b = 0.94, P < 0.0001, 95% CI: 0.61 to 1.27). Unilateral CS as measured by BCS was significantly associated with 10-2 VF MD ( r2 = 0.27, P = 0.006, 95% CI: 3.7 to 20.6), but not with 24-2 VF MD ( P = 0.151). CONCLUSION: This study suggests that a free, rapid home CS test correlates with glaucomatous macular damage as measured by 10-2 VF.

Association of Patterns of Glaucomatous Macular Damage With Contrast Sensitivity and Facial Recognition in Patients With Glaucoma.

Importance: Facial recognition is a critical activity of daily living that relies on macular function. Glaucomatous macular damage may result in impaired facial recognition that may negatively affect patient quality of life. Objective: To evaluate the association of patterns of glaucomatous macular damage with contrast sensitivity and facial recognition among patients with glaucoma. Design, Setting, and Participants: In this prospective cohort study at a single tertiary care center, 144 eyes of 72 consecutive patients with glaucoma with good visual acuity (20/40 or better in each eye) were studied. Data were collected from March to April 2019. Exposures: Eyes with macular damage were categorized as having focal, diffuse, or mixed (focal and diffuse) damage based on optic disc and macular spectral-domain optical coherence tomography and 10-2 visual field (VF) damage. Only eyes with focal or diffuse damage were included. Higher-acuity and lower-acuity eyes were determined by 10-2 VF mean deviation (MD). Facial disability was defined as facial recognition scores at the 2% level of normal participants. Main Outcomes and Measures: (1) Monocular contrast threshold as measured by the Freiburg Visual Acuity and Contrast Test and (2) binocular facial recognition as measured by the Cambridge Face Memory Test. Results: Of the 72 included patients, 49 (68%) were White and 41 (57%) were female, and the mean (SD) age was 67.0 (11.6) years. Eyes with diffuse damage had greater contrast impairment compared with eyes with focal damage (ß = -0.5; 95% CI, -0.6 to -0.4; P < .001) after adjusting for 10-2 VF MD, 24-2 VF MD, age, presence of an early cataract, and number of drops. Similarly, Cambridge Face Memory Test scores were significantly lower in patients with diffuse rather than focal macular damage, regardless of eye (better-seeing eye: ß = 10.0; 95% CI, 2.0 to 18.2; P = .001; worse-seeing eye: ß = 5.5; 95% CI, 0.8 to 10.0; P = .23). Relative risk of facial disability was greater for patients with diffuse but not focal macular damage in the better-seeing eye (relative risk, 86.2; 95% CI, 2.7 to 2783.3; P = .01). Conclusions and Relevance: In this cohort study, diffuse rather than focal glaucomatous macular damage was associated with diminished facial recognition and contrast sensitivity. Evaluation of macular optical coherence tomography and 10-2 VF and resultant detection of diffuse macular damage may help minimize glaucoma-related visual disability.

Macular Damage in Glaucoma is Associated With Deficits in Facial Recognition.

PURPOSE: This report examines the relationship between glaucomatous macular damage and facial recognition. In addition, it assesses the role of contrast sensitivity (CS) as an intermediary step in the causal pathway between macular damage and impairment of facial recognition. DESIGN: Prospective cross-sectional study. METHODS: This study was conducted in a single tertiary care center. The study population included 144 eyes of 72 participants with a diagnosis of open angle glaucoma in one or both eyes and a visual acuity of 20/40 or better in each eye. The presence or absence of macular damage was determined by comparing corresponding regions of the retinal nerve fiber layer and the retinal ganglion cell layer with spectral-domain optical coherence tomography with the 10-2 visual field (VF). Better and worse eye was determined by 10-2 VF mean deviations (MDs). Interventions were 1) macular function as measured by 10-2 VF and 2) CS as measured by the Freiburg Visual Acuity and Contrast Test (FrACT). The primary outcome measure was the Cambridge Face Memory Test (CFMT) score. RESULTS: Regardless of eye, there was a significant correlation between facial recognition and 10-2 VF MD (P < .0001 better, worse eye). The 10-2 VF MD remained a significant predictor of facial recognition after adjusting for potential confounders including glaucoma severity, CS, age, and visual acuity (P = .004 better eye, P = .019 worse eye). CONCLUSIONS: Even with good central visual acuity, patients with glaucomatous macular damage exhibit diminished facial recognition, which is partly mediated through diminished CS.

Diffuse Macular Damage in Mild to Moderate Glaucoma Is Associated With Decreased Visual Function Scores Under Low Luminance Conditions.

PURPOSE: Glaucoma patients commonly report increasing visual problems under low luminance or glare conditions, yet there is limited understanding of the structural basis of visual functional losses. This report examines the relationship between glaucomatous macular damage, assessed using structure-function correlation, and visual difficulty under low luminance conditions, as measured by Low Luminance Questionnaire (LLQ). DESIGN: Observational cohort study. METHODS: Setting: Tertiary care referral center. PARTICIPANTS: A total of 252 eyes of 126 participants with mild or moderate open-angle glaucoma (24-2 mean deviation [MD] better than -12 dB) selected from a consecutive sample. PREDICTOR: Focal and diffuse macular defects were identified based on corresponding abnormal regions on probability maps from spectral-domain optical coherence tomography (SDOCT) optic disc and macular cube scans, and 10-2 and 24-2 visual fields (VF). MAIN OUTCOME MEASURE: LLQ scores. RESULTS: Eighty-two of the 126 (65%) better eyes (defined by 24-2 VF MD) had evidence of macular damage, while the remaining 44 did not have macular damage. Of the 82 with damage, 33 (40%) had diffuse damage and 49 (60%) had focal damage. After adjusting for 24-2 MD and age in the multivariable regression, diffuse macular damage remained a significant predictor of the LLQ subscales "difficulty with extreme lighting" (P = .0024), ''difficulty with low lighting" (P = .037), and "diminished mobility"; (P = .042). In contrast, there was no significant difference in LLQ scores in any subscale between participants with focal macular damage and those without macular damage. CONCLUSION: Mild diffuse glaucomatous macular damage, as detected by abnormal topographic regions on measures of structure and function, is associated with decreased LLQ scores.