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Purpose: To determine the test-retest reliability and diagnostic accuracy of a binocular optical coherence tomography (OCT) prototype (Envision Diagnostics, El Segundo, California, USA) for pupillometry., Design: Assessment of diagnostic reliability and accuracy., Methods: Fifty participants with relative afferent pupillary defects (RAPDs) confirmed using the swinging flashlight method (mean age 49.6 years) and 50 healthy control subjects (mean age 31.3 years) were examined. Participants twice underwent an automated pupillometry examination using a binocular OCT system that presents a stimulus and simultaneously captures OCT images of the iris-pupil plane of both eyes. Participants underwent a single examination on the RAPDx (Konan Medical, Irvine, California, USA), an automated infrared pupillometer. Pupil parameters including maximum and minimum diameter, and anisocoria were measured. The magnitude of RAPD was calculated using the log of the ratio of the constriction amplitude between the eyes. A pathological RAPD was above ±0.5 log units on both devices., Results: The intraclass correlation coefficient was >0.90 for OCT-derived maximum pupil diameter, minimum pupil diameter, and anisocoria. The RAPDx had a sensitivity of 82% and a specificity of 94% for detection of RAPD whereas the binocular OCT had a sensitivity of 74% and specificity of 86%. The diagnostic accuracy of the RAPDx and binocular OCT was 88% (95% confidence interval 80%-94%) and 80% (95% confidence interval 71%-87%) respectively., Conclusions: Binocular OCT-derived pupil parameters had excellent test-retest reliability. The diagnostic accuracy of RAPD was inferior to the RAPDx and is likely related to factors such as eye movement during OCT capture. As OCT becomes ubiquitous, OCT-derived measurements may provide an efficient method of objectively quantifying the pupil responses., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Phosphenes are frequently reported by patients irradiated in the head and neck area. The aim of the present study was to characterize and investigate potential mechanisms of proton beam therapy (PBT)-induced phosphenes in a large population of patients undergoing PBT for ocular tumors., Design: Prospective cohort study., Methods: Consecutive patients who underwent PBT in a single center were included. Immediately after the first session, all patients completed a questionnaire collecting information about the presence of phosphenes as well as their color, shape, and duration. Patient, tumor and treatment characteristics (dose volume histograms) were also collected., Results: Among the 474 patients included, 62.8% reported phosphenes during the first session of PBT. Reported colors were mainly blue-violet (70.5%) and white (14.1%). The prevalence of phosphenes was higher in younger patients (P = .003); other patient or ocular characteristics were not associated with the occurrence of phosphenes. Irradiation of the macula (P < .001) and/or optic disc (P < .001) were significantly associated with the presence of phosphenes, whereas blue-violet color was only associated with young age and irradiation of macular area (P = .04). Pupillary constriction was reported for 57.1% of patients with phosphenes vs 18.5% of patients without (P < .001). Blue-violet phosphenes (P < .001) and irradiation of macula (P = .001) were statistically associated with pupillary constriction., Conclusions: The present study reported a high rate of phosphenes in patients irradiated by PBT for ocular tumor. Their blue-violet color and their association with a pupillary constriction probably indicates the stimulation of S-cones and retinal ganglion cells that reflects the activation of the afferent visual pathway., (Copyright © 2018 Elsevier Inc. All rights reserved.)

A 55-year-old HIV-positive man presented with acute vision loss in the right eye and altered mental status. Ophthalmic evaluation revealed light perception vision OD with a right relative afferent pupillary defect, conjunctival chemosis, large mutton-fat keratitic precipitates, and diffuse cream-colored vitreous cells. Magnetic resonance imaging of the brain and orbit with and without contrast with fat saturation showed choroidal thickening OD, multifocal deep periventricular and deep ganglionic enhancing lesions, and a suprasellar mass. Brain biopsy showed diffuse large B-cell lymphoma. Intrathecal chemotherapy with methotrexate and cytarabine and whole brain radiation therapy failed. His mental status deteriorated. He developed pancytopenia, neutropenic fever, and septic shock and subsequently expired under palliative care., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: To study the ocular phenotype of Pierson syndrome and to increase awareness among ophthalmologists of the diagnostic features of this condition., Design: Retrospective, observational case series., Methods: A multicenter study of 17 patients with molecularly confirmed Pierson syndrome. The eye findings were reviewed and compared to pertinent findings from the literature., Results: The most characteristic ocular anomaly was microcoria. A wide range of additional abnormalities were found, including posterior embryotoxon, megalocornea, iris hypoplasia, cataract, abnormal lens shape, posterior lenticonus, persistent fetal vasculature, retinal detachment, variable axial lengths, and glaucoma. There was high interocular and intrafamilial variability., Conclusions: Loss-of-function mutations in laminin beta2 (LAMB2) cause a broad range of ocular pathology, emphasizing the importance of laminin beta2 in eye development. Patients with Pierson syndrome can initially present with ocular signs alone. In newborns with marked bilateral microcoria, Pierson syndrome should be considered and renal function investigated.

Purpose: Pituitary adenomas are the most common cause of an optic chiasmal syndrome, and treatment of these lesions is considerably different from the treatment of most of the other lesions in this region. Although the diagnosis of a pituitary adenoma is usually inferred from the results of neuroimaging, lesions other than pituitary adenomas can have an appearance that suggests an adenoma. The objective of our study was to determine whether there are clinical findings that suggest a lesion producing a chiasmal syndrome is something other than a pituitary adenoma., Design: Retrospective, case-controlled, analysis of medical record data., Methods: The records of the Neuro-Ophthalmology Unit of the Wilmer Eye Institute were searched for patients with a chiasmal syndrome who had been evaluated before treatment and for whom pathologic or laboratory confirmation of the etiology was available. Presenting clinical features of these patients were recorded, and analyses with both a single variable and multiple variables were performed to determine whether there were any features that could identify with a high degree of probability the etiology of the lesion producing the syndrome., Results: The search revealed 149 patients who met the inclusion criteria, including 90 patients with pituitary adenomas and 59 patients with other lesions. Variables that were highly suggestive of an etiology other than pituitary adenoma included symptomatic visual loss, younger age, unilateral optic disk pallor, a relative afferent pupillary defect, and an absolute or a complete visual field defect or one was greater inferiorly than superiorly., Conclusion: Although no single clinical feature can be used to determine the specific nature of a lesion that produces an optic chiasmal syndrome, certain features are highly suggestive of an etiology other than pituitary adenoma. When these features are present, the likelihood that a suprasellar lesion is a pituitary adenoma is much lower, regardless of the appearance on neuroimaging.

Purpose: To report two cases of progressive outer retinal necrosis occurring in immunocompetent individuals after treatment with corticosteroids for presumed optic neuropathy., Design: Observational case report., Setting: University-based tertiary eye hospital., Methods: Retrospective review of existing clinical records., Results: Two patients were treated empirically with systemic corticosteroids for suspected inflammatory papillopathy. Subsequently, both were diagnosed with necrotizing herpetic retinitis with features of progressive outer retinal necrosis. Anterior chamber paracentesis confirmed varicella-zoster infection. Both patients were human immunodeficiency virus negative; one patient with rheumatoid arthritis was taking etanercept. Both became completely blind in one eye despite intensive treatment with antiviral medication intravenously and intravitreally., Conclusions: Progressive outer retinal necrosis is not confined to patients with underlying severe immunodeficiency, such as acquired immune deficiency syndrome. Initial treatment of acute, unexplained vision loss with systemic corticosteroids may lead to catastrophic visual loss in patients with evolving necrotizing herpetic retinopathy.

A 28-year-old woman presented with painful unilateral left visual loss, impaired color vision, left afferent pupillary defect, and normal ocular fundus. Although optic neuritis was first suspected, visual fields disclosed a junctional scotoma related to chiasmal demyelination, due to a probable multiple sclerosis.

Purpose: To describe a simple technique that detects a subtle relative afferent pupillary defect., Design: Observational report., Methods: With the patient seated at a slit lamp and the slit illumination turned off, a transilluminator light is swung back and forth between the eyes. The examiner observes the direct pupil light response and the consensual light response under slit-lamp magnification., Conclusion: Using slit-lamp magnification, the examiner may observe small amplitudes of pupillary movement in the evaluation of a relative afferent pupillary defect.

Purpose: To report a case of anterior ischemic optic neuropathy after a trans-Atlantic airplane journey., Design: An observational case report., Methods: A 48-year-old healthy man presented with severe visual loss in his left eye within 12 hours after a 15-hour-long trans-Atlantic airplane flight. The patient underwent slit-lamp examination, funduscopy, fluorescein angiography, automated perimetry, and various blood examinations., Results: Visual acuity was LE: 20/30, and a mild left eye relative afferent pupillary defect was noted. Funduscopic evaluation revealed an edematous optic disk in the left eye with a para-diskal retinal hemorrhage. Fluorescein angiography revealed a leaking optic disk, and perimetry showed an inferior hemi-field scotoma. Anterior ischemic optic neuropathy was diagnosed., Conclusion: Prolonged airplane travel may compromise vision either by a thromboembolic mechanism caused by prolonged immobilization or by a vasospasm mechanism induced by the low oxygen pressure during the flight.

Purpose: To report a case of tubercular endophthalmitis simulating retinoblastoma., Methods: Case report. An 8-year-old female presented with a history of complete loss of vision and a white pupillary reflex in the left eye of 3 month's duration., Results: Retinoblastoma could not be excluded on the basis of clinical examination and relevant investigational studies. In the left eye, a computed tomography (CT) scan demonstrated a large vitreous mass with foci of calcification. Enucleation in the left eye was performed, and histopathological examination revealed a chronic granulomatous endophthalmitis and acid-fast bacilli consistent with tubercular pathology., Conclusion: This case illustrates that tubercular endophthalmitis with leukocoria and a vitreous mass containing focal calcification may simulate retinoblastoma.

Purpose: To present the magnetic resonance imaging findings for a patient with traumatic oculomotor nerve injury., Methods: We examined a patient with a right pupil-involving oculomotor nerve palsy after severe closed head trauma., Results: Magnetic resonance imaging of the brain demonstrated marked signal hypointensity on gradient-echo T2*-weighted images consistent with hemorrhage at the midbrain exit site of the right oculomotor nerve., Conclusions: Distal fascicular damage or partial rootlet avulsion is a mechanism of injury in some traumatic oculomotor nerve palsies. Gradient-echo T2*-weighted magnetic resonance imaging is the most sensitive method to detect hemorrhagic changes associated with shearing injury.

We used crossed polarized neutral density filters to quantitate relative afferent pupillary defects. To prove reproducibility, the relative afferent pupillary defects of 20 patients were measured with this technique by two independent observers. There was no statistically significant difference between the two measurements. Relative afferent pupillary defects as low as 0.03 log unit were easily measured. These filters offer a convenient and reproducible technique that is more sensitive than the use of neutral density filters.