Visual Outcomes in Surgically Treated Intracranial Meningiomas.

BACKGROUND: Intracranial meningiomas that arise from the medial sphenoid ridge, anterior clinoid process, tuberculum sellae, or planum sphenoidale often impair vision by compressing the optic nerves and optic chiasm. Although many studies have reported visual outcome following surgery for these tumors, documentation has often been incomplete and not validated by patient self-report. METHODS: Retrospective study of 40 patients drawn from a single, academic, medical center. We used a unique method of assessing visual outcome based on whether the change in visual function affected the preoperatively better-sighted or worse-sighted eye in the belief that this method would correlate with effects on activities of daily living (ADL). To elicit patient self-reports of those effects, we conducted telephone interviews of 25 patients with a standard questionnaire. We also assessed putative ophthalmic, imaging, and surgical predictors of visual outcome. RESULTS: Visual improvement occurred in 61% of patients with preoperative monocular visual dysfunction, but only 22% of patients reported improvement in their ability to conduct ADL, and 17% lost vision. Visual outcomes were better in patients with preoperative binocular visual dysfunction, where visual improvement occurred in 73% and no patient lost vision in the preoperatively better-sighted eye. However, only 27% of patients with preoperative binocular visual dysfunction reported improvement in their ability to conduct ADL. Long duration of vision impairment, presence of optic disc pallor, large tumor size, and imaging-based preoperative optic canal involvement did not preclude a favorable visual outcome. Aggressive surgical reduction in displacement of the optic nerves was not necessary to obtain a favorable visual outcome and sometimes led to an unfavorable visual outcome. CONCLUSIONS: In this study, surgery often improved vision, especially in patients with preoperative binocular visual dysfunction. But patients indicated that the effect on their ability to perform ADL was more modest. Moreover, 17% of patients with preoperative monocular visual dysfunction lost vision in the only affected eye, often to a considerable degree. In those patients, surgery would be justified primarily to relieve the concern of having a large brain tumor and to prevent tumor growth. Preoperative ophthalmic and imaging features poorly predicted visual outcomes. Favorable visual outcomes occurred without aggressive surgical debulking of the tumors.

PATIENTS WITH EPIRETINAL MEMBRANES DISPLAY RETROGRADE MACULOPATHY AFTER SURGICAL PEELING OF THE INTERNAL LIMITING MEMBRANE.

PURPOSE: Intraretinal cystoid spaces are commonly found after surgical peeling of epiretinal membranes. In this study, we explored whether these cysts were associated with ganglion cell loss and thus might be a manifestation of retrograde maculopathy. The latter is a nonvascular edema with a characteristic morphology that is often found in the inner nuclear layer (INL) of patients with optic neuropathy. METHODS: In this retrospective case series, we identified consecutive patients who underwent surgical epiretinal membrane peeling. We determined the frequency of microcystic macular edema (MME), defined by vertical cystoid spaces in the INL, and we measured the thickness of individual macular layers before and after surgery. RESULTS: Epiretinal membrane peeling resulted in an improvement of visual acuity and a reduction of retinal thickness by about 15%. In total, 35% of patients with MME before surgery showed no sign of MME postoperatively, whereas edema persisted after surgery in 65% of patients. Interestingly, 29% of the patients without MME before surgery developed MME after surgery. Overall, we found MME in 35% of patients before peeling and in 42% after peeling. After surgery, the mean ganglion cell layer thickness was reduced compared with healthy control eyes. Ganglion cell layer thickness correlated inversely with thickness of the INL. Compared with patients without MME, individuals with MME had a thinner ganglion cell layer and a thicker INL in the affected eye. CONCLUSION: Our findings indicate that peeling of epiretinal membranes and internal limiting membranes is associated with atrophy of ganglion cells and thickening of the INL. The latter is associated with the presence of MME. Altogether, we assume that surgical treatment of epiretinal membranes induces a variant of a retrograde maculopathy.

Accuracy and Reliability of a Handheld, Nonmydriatic Fundus Camera for the Remote Detection of Optic Disc Edema.

BACKGROUND: Optic disc edema can be an important indicator of serious neurological disease, but is poorly detected using the direct ophthalmoscope. Portable fundus photography may overcome this difficulty. INTRODUCTION: The purpose of this study was to determine the sensitivity and specificity of a handheld, nonmydriatic fundus camera for the detection of optic disc edema. MATERIALS AND METHODS: Retrospective review of nonmydriatic optic disc photographs taken with a portable fundus camera (Pictor Plus; Volk Optical, Mentor, OH) from the University of Michigan Neuro-Ophthalmology Clinics. We included 103 consecutive eyes with optic disc edema and 103 consecutive eyes without optic disc edema of 109 patients. Four masked neuro-ophthalmologists graded a single photograph of each optic disc presented in randomized order and documented the presence of optic disc edema. Sensitivity and specificity of graders' photographic interpretation was compared with clinical examinations. Reliability of assessments within and between graders was determined using kappa statistics. RESULTS: The sensitivity and specificity for detection of optic disc edema were 71.8-92.2% and 81.6-95.2%, respectively. Photos were found to be ungradable in 0-8.3% of cases. The intergrader reliabilities ranged from 0.60 [95% confidence interval (CI): 0.52-0.67] to 0.72 (95% CI: 0.66-0.77). Intragrader reliability ranged from 0.76 (95% CI: 0.63-0.92) to 0.82 (95% CI: 0.69-0.95). DISCUSSION: Photographs taken with portable, nonmydriatic technology met threshold sensitivity and specificity for remote screening for optic disc edema when performed by most, but not all graders. Reliability between graders was moderate-strong and strong within individual providers. CONCLUSIONS: Portable photography holds promise for use in remote screening of optic disc edema.

Detection of Relative Afferent Pupillary Defects Using Eye Tracking and a VR Headset.

Purpose: The purpose of this study was to assess the feasibility of detecting relative afferent pupillary defects (RAPDs) using a commercial virtual reality headset equipped with an eye tracker. Methods: This is a cross-sectional study in which we compare the new computerized RAPD test with the traditional clinical standard using the swinging flashlight test. Eighty-two participants including 20 healthy volunteers aged 10 to 88 years were enrolled in this study. We present a bright/dark stimulus alternating between the eyes every 3 seconds using a virtual reality headset, and we simultaneously record changes in pupil size. To determine the presence of an RAPD, we developed an algorithm analyzing the pupil size differences. For the assessment of the performance of the automated and the manual measurement a post hoc impression based on all available data is created. The accuracy of the manual clinical evaluation and the computerized method is compared using confusion matrices and the gold standard of the post hoc impression. The latter is based on all available clinical information. Results: We found that the computerized method detected RAPD with a sensitivity of 90.2% and an accuracy of 84.4%, as compared to the post hoc impression. This was not significantly different from the clinical evaluation with a sensitivity of 89.1% and an accuracy of 88.3%. Conclusions: The presented method offers an accurate, easy to use, and fast method to measure an RAPD. In contrast to today's clinical practice, the measures are quantitative and objective. Translational Relevance: Computerized testing of Relative Afferent Pupillary Defects (RAPD) using a VR-headset and eye-tracking reaches non-inferior performance compared with senior neuro-ophthalmologists.

Expanding Genotype/Phenotype Correlation in 2p11.2-p12 Microdeletion Syndrome.

Chromosomal abnormalities on the short arm of chromosome 2 in the region p11.2 have been associated with developmental delay, intellectual disability, facial anomalies, abnormal ears, skeletal and genital malformations. Here we describe a patient with a de novo interstitial heterozygous microdeletion on the short arm of chromosome 2 in the region p11.2-p12. He presents with facial dysmorphism characterized by a broad and low root of the nose and low-set protruding ears. Clinical examinations during follow-up visits revealed congenital pendular nystagmus, decreased visual acuity and psychomotor development disorder including intellectual disability. The heterozygous 5 Mb-microdeletion was characterized by an array CGH (Comparative Genomic Hybridization) analysis. In the past two decades, nine patients with microdeletions in this region have been identified by array CGH analysis and were reported in the literature. All these patients show psychomotor development disorder and outer and/or inner ear anomalies. In addition, most of the patients have mild to severe intellectual disability and show facial malformations. We reviewed the literature on PubMed and OMIM using the gene/loci names as search terms in an attempt to identify correlations between genes located within the heterozygous microdeletion and the clinical phenotype of the patient, in order to define a recognizable phenotype for the 2p11.2p12 microdeletion syndrome. We discuss additional symptoms that are not systematically present in all patients and contribute to a heterogeneous clinical presentation of this microdeletion syndrome.

Homonymous hemianopia from infarction of the optic tract and lateral geniculate nucleus in deep cerebral venous thrombosis.

A 20-year-old man developed right homonymous hemianopia, hemiparesis, and hemisensory loss from deep cerebral venous thrombosis in the setting of high altitude. Approximately 3 months later, brain MRI showed encephalomalacia of the left optic tract and lateral geniculate nucleus, as well as signal abnormalities of the internal capsule and posterolateral thalamus. Homonymous hemianopia has previously been described in 1 case after deep cerebral venous thrombosis but without detailed neuroimaging features.

Differences in morphology and visual function of myelin oligodendrocyte glycoprotein antibody and multiple sclerosis associated optic neuritis.

BACKGROUND: Myelin oligodendrocyte glycoprotein immunoglobulin G associated optic neuritis (MOG-ON) is a recently described entity. Recent studies have shown that MOG-ON has a more severe clinical presentation than classic optic neuritis (ON). OBJECTIVE: This study aimed to define morphological characteristics of MOG-ON, correlate these with clinical characteristics and compare them with multiple sclerosis associated ON (MS-ON) and healthy controls (CTRL). METHODS: In a retrospective study, we included MOG-ON and MS-ON patients seen between 2011 and 2018 at the University Hospital Bern. Data from clinical examination, perimetry, and optical coherence tomography (OCT) were analyzed. RESULTS: A total of 66 eyes of 43 patients were included; 22 MS-ON and 33 CTRL eyes were sex- and age-matched to 11 MOG-ON eyes. We found significantly worse visual acuity at nadir, but better recovery and thinner global peripapillary retinal nerve fiber layer thickness in MOG-ON patients compared to MS-ON patients. Both groups exhibited irregular thinning of the macular ganglion cell layer. Furthermore, the visual acuity and visual field parameters correlated to retinal layer thickness only in MOG-ON eyes. CONCLUSION: In comparison to MS-ON, MOG-ON is associated with more prominent acute vision loss and more pronounced global thinning of the pRNFL. Both entities result in similar final visual acuity and atrophy of the macular ganglion cell layer.

Evaluation of diagnostic criteria and red flags of myelin oligodendrocyte glycoprotein encephalomyelitis in a clinical routine cohort.

AIMS: Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been proposed to define "MOG encephalomyelitis" (MOG-EM), with published diagnostic and "red flag" criteria. We aimed to evaluate these criteria in a routine clinical setting. METHODS: We retrospectively analyzed patients with borderline/positive MOG-IgG and applied the diagnostic and red flag criteria to determine likelihood of MOG-EM diagnosis. Para-/clinical parameters were described and analyzed with chi-square test. RESULTS: In total, 37 patients fulfilled MOG-EM diagnostic criteria (female-to-male ratio: 1.6:1, median onset age: 28.0 years [IQR 18.5-40.5], n = 8 with pediatric onset). In 24/37, red flags were present, predominantly MOG-IgG at assay cutoff and/or MRI lesions suggestive of multiple sclerosis (MS). As proposed in the consensus criteria, these patients should rather be described as "possible" MOG-EM. Of these, we classified 13 patients as "unlikely" MOG-EM in the presence of the red flag "borderline MOG-IgG" with negative MOG-IgG retest or coincidence of ≥1 additional red flag. This group mainly consisted of patients diagnosed with MS (n = 11). Frequency of cerebrospinal fluid (CSF-)-specific oligoclonal bands (OCB) is significantly lower in definite vs possible and unlikely MOG-EM (P = .0005). CONCLUSION: Evaluation of diagnostic and red flag criteria, MOG-IgG retesting (incl. change of assay), and CSF-specific OCB are relevant in clinical routine cohorts to differentiate MOG-EM from MS.

Homonymous hemiatrophy of ganglion cell layer from retrochiasmal lesions in the visual pathway.

OBJECTIVE: To determine the temporal evolution, morphology, and frequency of macular ganglion cell atrophy in patients with retrochiasmal lesions of the visual pathway. METHODS: In a consecutive retrospective case series, we identified 47 patients with homonymous hemianopia and accessible macular optical coherence tomography scans. We estimated the time of lesion onset and the location of the lesion within the afferent visual pathway. Using semiautomatic layer segmentation, we determined ganglion cell layer thickness in areas projecting to the side of the retrochiasmal lesion and compared it with ganglion cell layer thickness on the healthy side. RESULTS: We found that retrochiasmal lesions at any level may be associated with an atrophy of ganglion cells. This atrophy respects the vertical midline through the fovea and thus the anatomic separation of the nasal and temporal visual field. The vertical line separating the affected from the unaffected side has significantly less tilt as compared with the disc-fovea angle. Lesions of the optic tract are associated with earlier macular ganglion cell atrophy than retrogeniculate lesions. Macular ganglion cell atrophy may be present in cases with normal peripapillary nerve fiber layer analysis and vice versa. CONCLUSIONS: Macular ganglion cell layer thickness shows a topographic hemiatrophy in retrochiasmal lesions, which manifests earlier for tract lesions than for retrogeniculate lesions. This additional examination of ganglion cell homonymous hemiatrophy has a higher sensitivity in detecting retrograde transsynaptic degeneration than the analysis of the peripapillary nerve fiber layer alone.

Retrograde Maculopathy in Patients With Glaucoma.

PURPOSE: Macular optical coherence tomography (OCT) analysis can be used for quantitative measures of optic nerve atrophy at a location far from the optic nerve head. This recently led to the finding of microcystic macular edema (MME), that is vacuolar inclusions in the macular inner nuclear layer, in some glaucoma patients. The involvement of individual retinal layers is yet unclear in glaucoma. In this study we systematically investigated glaucoma-induced changes in macular layers to evaluate whether glaucoma-associated damage extends beyond the macular ganglion cell layer. PATIENTS AND METHODS: We included 218 consecutive patients and 282 eyes with confirmed primary open-angle glaucoma or pseudoexfoliation glaucoma, and macular OCT in a cross-sectional observational study. Eyes were screened for presence of MME. Thickness of individual retinal layers was determined using a semiautomatic segmentation algorithm. Peripapillary nerve fiber layer thickness and mean defect in visual field testing were extracted from OCT and medical records, respectively. Results were compared with a small group of eyes with no apparent glaucoma. RESULTS: We found MME in 5 eyes from 5 primary open-angle glaucoma patients and 3 eyes of 3 pseudoexfoliation glaucoma patients (2.8%). MME was confined to the inner nuclear layer in a perifoveal ring and was associated with thinning of the ganglion cell layer and thickening of the macular inner nuclear layer. Glaucoma eyes without MME showed a significant inverse correlation of inner nuclear layer thickness with glaucoma severity. CONCLUSIONS: Glaucomatous damage leads to a gradual thickening of the inner nuclear layer, which leads to MME in more severe glaucoma cases. These changes, along with nerve fiber loss and ganglion cell loss, may be summarized as glaucoma-associated retrograde maculopathy.

Persistent diplopia and superior oblique muscle dysfunction following dissection of the orbital periosteum in cranial base surgery.

BACKGROUND/AIMS: Persistent diplopia secondary to a fourth cranial nerve palsy is poorly documented after open cranial base surgery. METHODS: Six cases of fourth cranial nerve palsy after cranial base surgery were drawn from the Neuro-Ophthalmology and Head and Neck Surgery Clinics at the University of Michigan from 2004 to 2012. RESULTS: Six patients developed diplopia and ocular misalignment in a pattern suggestive of superior oblique palsy following dissection of the medial orbital periosteum as part of a surgical approach to the anterior cranial base. Among the four patients in whom follow-up examination was available, the misalignment improved spontaneously in three patients and was stable in the fourth patient, but did not completely resolve in any patient. CONCLUSIONS: This sparsely documented phenomenon is likely caused by dysfunction of the superior oblique muscle, possibly the result of malposition of the trochlea after spontaneous reattachment of the periosteum. Special factors such as invasive tumours, repeated surgeries of this nature, prior radiation, or chemical cementing material that adversely affects wound healing may be contributory.

A case of purtscher-like retinopathy following a motor vehicle accident.

PURPOSE: To describe a case of Purtscher-like retinopathy following seat belt compression of the chest in a motor vehicle accident. METHODS: Case report. RESULTS: A 53-year-old woman developed Purtscher-like retinopathy following compression of the chest by her seat belt in a motor vehicle accident. Observation was recommended, and on follow-up examination at 8 weeks her vision, fluorescein angiography, and optical coherence tomography had returned to baseline. CONCLUSION: Seat belts may produce chest compression to induce a Purtscher-like retinopathy in varying degrees associated with the severity of the accident and subsequent compressive forces of the seat belt.

Adverse events after pars plana vitrectomy among medicare beneficiaries.

OBJECTIVES: To assess the complication rates of pars plana vitrectomy (PPV) among older Americans and to determine whether rates of adverse events and additional operations have changed during the past decade. METHODS: Claims data were reviewed to identify all adults aged 68 years or older in the 5% Medicare sample who underwent their first PPV during 1994-1995, 1999-2000, and 2004-2005. One-year rates of severe complications (endophthalmitis, suprachoroidal hemorrhage, or retinal detachment), less severe complications, receipt of an additional operation, and blindness were calculated and compared among the 3 groups using Cox regression. Analyses were adjusted for prior adverse events (during the previous 3 years), demographic characteristics, and comorbid conditions. RESULTS: The 1994-1995, 1999-2000, and 2004-2005 cohorts had 3263, 5064, and 5263 patients, respectively. The 1-year severe complication rates did not differ among the 3 groups (range, 4.8%-5.5%). The hazard of a less severe complication or an additional operation was higher in the 2004-2005 cohort than in the earlier cohorts (P < .05 for all comparisons). The hazard of endophthalmitis was higher in black individuals (P = .07) and those of other races (P = .02) than in white patients. CONCLUSIONS: During the past decade, rates of severe complications after PPV remained stable, but rates of less severe complications and subsequent operations increased. Future studies should explore the potential factors that explain these changes and the alarming elevated incidence of post-PPV endophthalmitis among nonwhite individuals.