Tomographic Predictors of Ectasia Development After Keratorefractive Surgery.

PURPOSE: The aim of this study was to identify the risk factors for postkeratorefractive surgery ectasia in unsuspicious tomographies and to report a new index coined diameter of peak locations (DPLs). METHODS: All patients who underwent keratorefractive surgery between 2011 and 2018 at Care-Vision Laser Centers, Israel, and later developed ectasia were included. For each ectasia case, 3 matched controls were selected. Demographic and preoperative, intraoperative, and postoperative data were collected. Multivariate analysis was performed to evaluate the interdependence of the variables. RESULTS: The retrospective study included 19 ectasia and 58 control eyes. There were no significant differences between the groups in ablation depth ( P = 0.73), preoperative spherical equivalent ( P = 0.12), percent tissue altered ( P = 0.71), residual stromal bed ( P = 0.73), and Ectasia Risk Score System ( P = 0.60). The anterior and the posterior symmetry index were significantly higher ( P < 0.001), and DPL was significantly tighter in the ectasia group ( P = 0.01). Binary multiple logistic regression found the symmetry index of the posterior cornea and DPL to be better predictors than age, percent tissue altered, Ectasia Risk Score System, residual stromal bed, and ablation depth. CONCLUSIONS: A higher posterior symmetry index and a tighter DPL are predictors of ectasia in patients with otherwise normal tomographies. A tight DPL implies a weak area in the cornea, which is thin and slightly bulging, increasing the risk of this area becoming ectatic.

Emergent cerebral venous stenting: A valid treatment option for fulminant idiopathic intracranial hypertension.

BACKGROUND: Fulminant idiopathic intracranial hypertension (FIIH) is characterized by rapid, severe, progressive vision loss and often treated surgically. Cerebral transverse venous stenting (CTVS) is efficacious in IIH patients, but emergent CTVS in FIIH is rarely reported. We present our experience with emergent CTVS in patients with FIIH. METHODS: Since 01/2019, an institutional protocol allowed emergent CTVS in FIIH patients with bilateral transverse sinus stenosis and gradient pressure > 15 on digital subtraction angiography (DSA). We retrospectively analyzed a prospective registry of all IIH patients with details of neurological and neuro-ophthalmological assessments before and after treatment, and subjective assessments of headache and tinnitus were made pre-and post-procedure. RESULTS: 259 IIH patients, including 49 who underwent CTVS, were registered. Among them, five female patients met inclusion criteria for FIIH and underwent emergent CTVS. FIIH patients were younger (18.8 ± 1.64 vs 27.7 ± 4.85, p < 0.01), mean BMI was lower (30.8 ± 10.57 vs 34.6 ± 4.3, p < 0.01), and lumbar puncture opening pressure higher (454 ± vs 361 ± 99.4, p < 0.01) than that of IIH patients. They presented with acute visual loss, severe headache, papilledema, significant bilateral transverse sinus stenosis on CT-venography, and mean dominant side gradient pressure of 26.4 ± 6.2 on DSA. CTVS was performed without significant complications, resulting in remarkable improvement in headache, optical coherence tomography, and visual fields within 1 week. At 1-year follow-up (four patients) and 6-month follow-up (1 patient), there was complete resolution of papilledema and headache, and marked improvement in visual acuity. CONCLUSIONS: In these patients, emergent-CTVS was a safe and effective treatment option for FIIH. Further evaluation is warranted.

A Consensus Statement on the Terminology for Automated Visual Field Abnormalities.

BACKGROUND: A multitude of terms have been used to describe automated visual field abnormalities. To date, there is no universally accepted system of definitions or guidelines. Variability among clinicians creates the risk of miscommunication and the compromise of patient care. The purposes of this study were to 1) assess the degree of consistency among a group of neuro-ophthalmologists in the description of visual field abnormalities and 2) to create a consensus statement with standardized terminology and definitions. METHODS: In phase one of the study, all neuro-ophthalmologists in Israel were asked to complete a survey in which they described the abnormalities in 10 selected automated visual field tests. In phase 2 of the study, the authors created a national consensus statement on the terminology and definitions for visual field abnormalities using a modified Delphi method. In phase 3, the neuro-ophthalmologists were asked to repeat the initial survey of the 10 visual fields using the consensus statement to formulate their answers. RESULTS: Twenty-six neuro-ophthalmologists participated in the initial survey. On average, there were 7.5 unique descriptions for each of the visual fields (SD 3.17), a description of only the location in 24.6% (SD 0.19), and an undecided response in 6.15% (SD 4.13). Twenty-two neuro-ophthalmologists participated in the creation of a consensus statement which included 24 types of abnormalities with specific definitions. Twenty-three neuro-ophthalmologists repeated the survey using the consensus statement. On average, in the repeated survey, there were 5.9 unique descriptions for each of the visual fields (SD 1.79), a description of only the location in 0.004% (SD 0.01), and an undecided response in 3.07% (SD 2.11%). Relative to the first survey, there was a significant improvement in the use of specific and decisive terminology. CONCLUSIONS: The study confirmed a great degree of variability in the use of terminology to describe automated visual field abnormalities. The creation of a consensus statement was associated with improved use of specific terminology. Future efforts may be warranted to further standardize terminology and definitions.

The Chiasmal Compression Index: An Integrative Assessment Tool for Visual Disturbances in Patients with Pituitary Macroadenomas.

OBJECTIVE: Visual status is routinely evaluated by neuro-ophthalmologic examination and computerized visual field (VF) tests in patients with chiasmal compression secondary to pituitary macroadenoma. Currently, no relevant data exists to accurately quantify the extent of optic apparatus compromise to further guide clinical decision-making. We aimed to assess for a possible quantitative correlation between optic chiasm geometric properties on magnetic resonance imaging (MRI) and VF deficits. METHODS: Visual assessments and concurrent MRI scans were retrospectively reviewed from patients treated for pituitary macroadenoma in a single medical institution. Chiasm width, chiasm minimal and maximal height, and chiasm angle were measured on MRI coronal plane images by 3 independent reviewers (for the sake of variability analysis). VF numerical summary parameters were also retrieved. RESULTS: A total of 30 patients were included in the final analysis. Average VF index was 70% (±30), and averaged mean deviation was 10.0 db (±9). Chiasm angle and width (which together represents the bending and stretching of the chiasm by the upward directed compression; both of which demonstrated high inter- and intraobserver agreement) showed strong correlation with VF loss. Chiasmal compression index derived from those parameters showed even stronger correlation. CONCLUSIONS: The strong correlation demonstrated by our results of this relatively simple radiologic measurement with VF status, despite the relatively small cohort, calls for further investigation in this promising direction, and may facilitate with basic assessment and clinical decision-making for patients with equivocal neuro-ophthalmologic evaluation, as well as with poor compliance.

Age-Related Clinical Presentation of MOG-IgG Seropositivity in Israel.

Introduction: Myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders (MOGAD) have been recognized over the past 10 years as distinct inflammatory, demyelinating diseases of the central nervous system (CNS). Antibodies against MOG are found mostly in patients with optic neuritis (ON), acute disseminated encephalomyelitis (ADEM), and aquaporin-4 antibody (AQP4-abs)-seronegative neuromyelitis optica spectrum disorders (NMOSD). However, data on the disease course and disability outcomes of these patients are scarce. Aim: To describe clinical and paraclinical features associated with MOG antibodies (abs) in a cohort of patients in Israel, and to assess baseline prognostic features of MOG-ab-associated diseases after a first acute demyelinating event. Methods: MOG-abs were identified in serum using a cell-based assay, and clinical data were collected from the patients' medical records. Results: Of 683 patients with demyelinating diseases tested for MOG-abs, 53 were positive (7.7%), with ON the most common presenting phenotype (68%). The age range of MOG-abs seropositive patients was 1-66 years, with increased prevalence in children (19% compared to 6.7% in adults) (p < 0.01). The highest prevalence of seropositivity was observed in children aged younger than 10 years (25.5%), followed by those aged 31-40 years (16.6%). Conclusions: MOGAD are distinct autoimmune diseases that occurs at all stages of life with a significantly higher prevalence in children; the main clinical presenting phenotype in the entire cohort is ON and young children most often presented with ON or ADEM. Our data highlight the need for repeated evaluation of MOG-abs in patients with acquired CNS demyelinating disorders, especially in children under 10 and adults between 31 and 40 years of age.

[TREATMENT DILEMMAS IN FULMINANT INTRACRANIAL HYPERTENSION].

INTRODUCTION: Fulminant intracranial hypertension is a rare, acute presentation of idiopathic intracranial hypertension with rapid and devastating visual loss that can lead to blindness. As vision deteriorates quickly and often irreversibly, prevention of further visual loss requires emergent treatment to decrease intracranial pressure. The case presented is that of an 18 years old male with new symptoms of headaches and visual obscurations that had rapid progressive visual loss with findings of severe bilateral swollen discs. Brain MRI and MRV ruled out intracranial mass and cerebral venous thrombosis. A lumbar puncture confirmed highly increased ICP. Due to the fulminant clinical presentation, he underwent an urgent fenestration of both optic nerves with improvement of vision in both eyes. The patient's clinical scenario accentuates the importance of prompt recognition of this rare disorder and the need for immediate surgical intervention to prevent further visual loss and blindness.

A rat model for acute rise in intraocular pressure: immune modulation as a therapeutic strategy.

PURPOSE: To establish a rat model of acute increase in intraocular pressure (IOP) and to investigate the therapeutic window for protection against death of retinal ganglion cells (RGCs) by vaccination with glatiramer acetate (Cop-1) or by treatment with brimonidine or MK-801. DESIGN: Animal study, laboratory investigation. METHODS: IOP was transiently increased in anesthetized Lewis rats by infusing normal saline (0.9%) into the anterior chamber of the eye for one hour. RGC survival was assessed one week and two weeks later by counting the RGCs retrogradely labeled with rhodamine dextran. MAIN OUTCOME MEASURES: RGC survival. RESULTS: IOP rose to 100 cm H(2)O (76 mm Hg) and returned to baseline after 24 hours. The RGC count decreased by 23% a week after the insult and by a further 7% after the second week. Vaccination with Cop-1 on the day of the insult prevented 50% of the IOP-induced RGC loss. Similar neuroprotection was achieved by daily intraperitoneal injections of brimonidine, but not with MK-801. CONCLUSIONS: A transient increase in IOP to 100 cm H(2)O causes death of RGCs in rats. A single immunization with Cop-1 or daily injections of brimonidine protected up to 50% of potentially doomed RGCs from IOP-induced death, suggesting that not all of the cell death in the untreated model results from the IOP insult directly, but that some of it is caused by insult-induced environmental cytotoxicity, which is unrelated to glutamate toxicity or at least to NMDA receptors. These findings can be applied immediately as a basis for acute glaucoma therapy.

[Intravitreal triamcinolone acetonide for diffuse diabetic macular edema--one year follow-up].

BACKGROUND: Macular edema is the main cause of visual impairment in diabetic patients. Its treatment is mainly based on laser photocoagulation. Intravitreal triamcinolone acetonide (TA) has recently been proposed as a new treatment for eyes with diabetic macular edema resistant to conventional laser photocoagulation. AIM: To evaluate the one year efficacy and safety of a single TA injection administered for diffuse diabetic macular edema unresponsive to prior laser treatment. DESIGN: Interventional case series. METHODS: Setup: University medical center out-patient clinic. PARTICIPANTS: Twenty-one patients with bilateral diffuse diabetic macular edema. INTERVENTION: A single intravitreal injection of triamcinolone acetonide. MAIN OUTCOME MEASURES: Visual acuity measured by ETDRS score, retinal area evaluation clinically and retinal thickness evaluation by Retinal Thickness Analysis (RTA) at 3 and 12 months following injection. Secondary outcomes were intraocular pressure control and cataract progression. RESULTS: Three months following injection, the mean improvement in visual acuity was 4.7 +/- 11.7 letters in the study group as compared to 0.2 +/- 11.4 in the control group (p = 0.18). No difference was noticed one year following injection. Clinical assessment of the retinal area of edema revealed a substantial difference between patients and controls at 3 months (p = 0.0006) and at one year (p = 0.05). RTA evaluation revealed improvement in retinal thickness solely at the 3 months exam. Four eyes developed high intraocular pressure and required treatment (p = 0.054). No difference in cataract progression was noted between the two groups (p = 0.69). CONCLUSIONS: Injection of TA may improve visual acuity for a limited time. Increased intraocular pressure is a frequent side effect. During a one year follow-up, no evidence of ocular toxicity was noted in eyes with diffuse diabetic macular edema.

T-cell-based vaccination for morphological and functional neuroprotection in a rat model of chronically elevated intraocular pressure.

Acute or chronic glaucoma is often associated with an increase in intraocular pressure (IOP). In many patients, however, therapeutic pressure reduction does not halt disease progression. Neuroprotection has been proposed as a complementary therapeutic approach. We previously demonstrated effective T-cell-based neuroprotection in experimental animals vaccinated with the synthetic copolymer glatiramer acetate (copolymer-1, Cop-1), a weak agonist of self-antigens. This study was undertaken to test different routes and modes of vaccination with Cop-1 as treatment modalities for protection against retinal ganglion cell (RGC) death caused by chronic elevation of IOP in rats, and to determine whether anatomical neuroprotection is accompanied by functional neuroprotection. In a chronic model of unilaterally high IOP, Cop-1 vaccination, with or without an adjuvant, protected rats against IOP-induced loss of RGCs by eliciting a systemic T-cell-mediated response capable of cross-reacting with self-antigens residing in the eye. In rats deprived of T cells, Cop-1 (unlike treatment with alpha2-adrenoreceptor agonists) was not protective of RGCs, substantiating the contention that its beneficial effect is not conferred directly but is T-cell-mediated. Pattern electroretinography provided evidence of functional protection. Thus, vaccination with adjuvant-free Cop-1 can protect RGCs from the consequences of elevated IOP in rats. This protection is manifested both morphologically and functionally. These findings can be readily implemented for the development of a therapeutic vaccination to arrest the progression of glaucoma.

Special report: Noninvasive multi-parameter functional optical imaging of the eye.

Advancement in the treatment of blindness depends on the development of new technologies that enable early detection, follow-up, and treatment of disease. The authors describe direct, noninvasive imaging of four parameters: blood flow, blood oximetry, metabolic state, and hidden vasculature, particularly capillaries. These are functional parameters of the retina known to be degraded by retinal disease. The new Retinal Function Imager (Optical Imaging, Ltd., Rehovot, Israel) can image all four parameters as intrinsic reflectance intensity differences over the retina's surface. During the past 2 decades, imaging of small optical signals has been a powerful tool for high-resolution functional mapping in the neocortex. In this article, this technology is applied to the retina and demonstrates a general tool for noninvasively probing retinal function in many modalities. Imaging functional changes before anatomic consequences arise holds promise as a powerful tool for early diagnosis and treatment of retinal disease.

Eyelid bleeding and atypical amyloidosis.

PURPOSE: To describe a case of atypical systemic amyloidosis in a patient who presented with recurrent subcutaneous bleeding of the eyelids and auricles. DESIGN: Observational case report. METHODS: A 52-year-old man with chronic cardiac failure, hypertension, weight loss, and impotence presented with recurrent bleeding of the eyelids and auricles, and was investigated for suspected amyloidosis. RESULTS: Biopsy specimens taken from the bone marrow, salivary gland, abdominal subcutaneous fat, and gastric mucosa, and eyelids were all negative for Congo red. Results of a conjunctival biopsy, which were returned after the patient's death, and cardiac muscle autopsy were positive for Congo red staining, indicating amyloidosis. CONCLUSION: The findings in this case show that the presentation of amyloidosis may be atypical. Ophthalmologists should consider a diagnosis of amyloidosis in patients with periorbital bleeding, even if biopsies are negative for Congo red. A conjunctival biopsy may be useful in establishing a diagnosis of primary systemic amyloidosis.

High-resolution functional optical imaging: from the neocortex to the eye.

Much work remains to be done to establish the clinical usefulness of the RFI for early diagnosis and treatment guidance. The discoveries obtained by functional optical imaging of the neocortex in the last 15 years and the recent RFI studies of the eyes of normal subjects and patients with diabetic retinopathy, glaucoma, and age-related macular degeneration suggest that functional optical imaging of the retina is likely to become a multi-modality powerful clinical tool.