Longitudinal optical coherence tomography indices in idiopathic intracranial hypertension.

Idiopathic intracranial hypertension (IIH) may result in optic nerve fiber loss and even atrophy. The timing of the optical coherence tomography (OCT) indices reaching the lowest point (nadir) and the factors that predict the patient's anatomical outcome are not known. We aimed to determine the timing and the factors that affect nadir retinal nerve fiber layer (RNFL) thickness. The medical records of 99 IIH patients who were treated from December 2009 to January 2020 were retrospectively reviewed. The mean RNFL thickness at presentation was 263.5 ± 106.4 µm. The mean time to nadir was 7.9 ± 6.3 months. The average RNFL and ganglion cell complex (GCC) thickness at the nadir were 92.6 ± 14.5 µm (47% showed thinning) and 77.9 ± 27.8 µm (70% showed thinning), respectively. The Frisén disc edema stage and average RNFL thickness at baseline correlated with a longer time to nadir, (r = 0.28 P = 0.003 and r = 0.24, P = 0.012, respectively). The nadir average RNFL thickness and the nadir average GCC thickness (r = 0.32, P = 0.001, r = 0.29, P = 0.002, respectively) correlated with the baseline visual field mean deviation. The final anatomical outcome of IIH episodes in this study resulted in RNFL and GCC thinning. The time to RNFL nadir and its values correlated with IIH severity at presentation.

Study in Patients With Unilateral Horner Syndrome Supports the Role of Müller's Muscle in the Eyelid-Dark Reflex.

Purpose: A previous study demonstrated upper eyelid retraction synchronized with pupil dilation following a transition from photopic to scotopic conditions. The current study aimed to evaluate the role of Müller's muscle as the efferent arm of this suggested reflex arc. Methods: A video scan of both eyes of patients with unilateral Horner syndrome was performed using optical coherence tomography infra-red mode to document the transition between photopic and scotopic conditions. The affected side with sympathetic denervation was the study group, whereas the contralateral unaffected side of the same patients served as the control group. The pupil diameter, upper eyelid margin-to-reflex distance 1, lower eyelid margin-to-reflex distance 2, and vertical palpebral fissure height were measured. The control group was compared to the healthy subjects of a previous study to verify any compensatory changes to the side contralateral to denervation. Results: Ten patients with unilateral Horner Syndrome were included in the study. Transitioning from photopic to scotopic conditions, the mean change in margin-to-reflex distance 1 in the study and control groups was 315 ± 276 µm (P < 0.05) and 723 ± 432 µm (P = 0.005), respectively. Margin-to-reflex distance 1 and palpebral fissure height were significantly higher in the control group both in photopic (P = 0.005 and P = 0.017, respectively) and scotopic conditions (P = 0.005 and P = 0.007, respectively). The change in margin-to-reflex distance 1 and palpebral fissure height following the transition from light to dark was significantly greater in the control group (P = 0.022). Conclusions: Reflexive eyelid retraction following a transition from photopic to scotopic conditions was significantly diminished in eyelids with sympathetic denervation compared with the unaffected contralateral side of the same patients. This study provides further evidence that the sympathetically innervated Müller's muscle serves as the efferent arm of this reflex.

Diplopia in the Younger Adult (≤65 Years Old) Compared With Older Adult (>65 Years Old) Population-Presentation, Progression, and Outcome.

BACKGROUND: Despite the impact of new-onset diplopia on the quality of life, there are few studies concerning new-onset diplopia in seniors. This study aimed to describe the epidemiology, etiology, prognosis, and outcome of different treatments in the older adults compared with younger adult patients presenting with new-onset binocular diplopia. METHODS: A retrospective chart review of patients ≥18 YO with new-onset binocular diplopia presenting between 2010 and 2021. Data collected included age at presentation, gender, duration of time since diplopia onset, imaging results, known trigger, etiology, treatment, and follow-up. RESULTS: Two hundred ten patients were included. Of them, 75 patients were ≤65 YO (35.7%, the "younger adult group") and 135 > 65 YO (64.3%, the "older adults group"). The common etiology in both groups was neurogenic (54.7% ≤ 65 vs 62.2% >65, P = 0.29). Cranial nerve palsies were more commonly microvascular in the older adults (96.0% vs 74.1%, P = 0.005), whereas tumor-related cranial nerve palsies were more frequent in younger adults (14.81% vs 2.04%, P = 0.03). A restrictive etiology was observed in 20% of younger adult compared with 11.1% of older adults group (P = 0.08). Sagging eye syndrome (SES) was the second most common etiology in the older adults group at 11.9%, compared with 1.3% in the younger adult group (P = 0.01). Decompensated phoria/tropia appeared in 16% of younger adult group compared with 11.9% of older adults (P = 0.4), with an obvious trigger (mostly cataract surgery) in the latter (80% older adults vs 20% younger adults, P = 0.019). Positive imaging findings were found in 46.7% of patients ≤65 compared with 25.3% of >65 (P = 0.01) and complete spontaneous resolution of diplopia was noted in 32.1% of the older adults compared with 11.8% of younger adults (P = 0.003). CONCLUSIONS: Neurogenic diplopia was the most common etiology for both groups, but is more prominent in the older adults. Noticeable findings in the older adults were SES diagnosis, identification of triggers for impaired fusion/diplopia, and a paucity of positive findings in imaging results. It is important to know these differences not only for managing seniors better, but also to minimize symptoms of binocular diplopia after lens-related procedures.

Prognostic Value of Optical Coherence Tomography Characteristics in Anterior Visual Pathway Meningiomas.

BACKGROUND: Anterior visual pathway meningiomas (AVPM) represent 2.5%-18% of all meningiomas. They may affect visual function, including visual acuity (VA) and visual field (VF). The principal modes of treatment are surgery and radiotherapy. The prognostic value of macular ganglion cell complex count (GCC) thickness has not been assessed in the literature thus far. The purpose of this study was to evaluate the prognostic value of pre-treatment optical coherence tomography (OCT) parameters (retinal nerve fiber layer and GCC) for visual outcomes in patients with AVPM. METHODS: We retrospectively reviewed the medical records of all patients with AVPM who were treated in the Sheba Medical Center between 2011 and 2020. Included were patients with valid data containing preintervention OCT findings on the CIRRUS device and a minimum follow-up of 6 months. Preintervention and postintervention data on comprehensive ophthalmic examinations and OCT parameters of the affected eyes were retrieved. The correlation between preintervention OCT parameters and the visual outcome was assessed. The patients were also divided into 2 groups according to preintervention GCC (thin vs normal), and the visual outcome was compared between groups. RESULTS: In total, 186 patients' medical records were analyzed, and 38 patients who met the inclusion criteria were included in the study (mean age at diagnosis 52.8 ± 12.2 years, 28 women). Twenty-nine patients had 1 affected eye, and 9 had bilateral insult. A higher preinterventional average GCC was associated with better VA at 6 months, 1 year, and 2 years after intervention (r = -0.5, P ≤ 0.004, 0.005, and 0.03, respectively). There was a significant correlation between preinterventional GCC and VF mean deviation 2 years after intervention (r = 0.7, P ≤ 0.001). The thinner the GCC, the more prominent was the change in VA from before intervention to 2 years after intervention ( P ≤ 0.008). Correction for multiple comparisons with the Benjamini-Hochberg procedure did not change the significance of our findings. CONCLUSIONS: OCT parameters (GCC) have a predictive value in AVPM. There is strong correlation between preinterventional GCC and VA shortly after the intervention. Although a thin GCC is generally considered a negative prognostic factor, improvement in clinical parameters was also evident in patients with thin GCC. The potential of improvement despite preinterventional GCC thinning can add to the clinical discussion of the prognosis, and therefore, we recommend the patients with AVPM to undergo OCT and to be advised that GCC thinning alone should not be used as a major criterion in deciding whether treatment should be pursued.

A Multidisciplinary Thyroid Eye Clinic: A One-stop Shop.

BACKGROUND: Little is known about the success of multidisciplinary thyroid eye disease (TED) clinic. OBJECTIVES: To present the characteristics, treatments, and outcomes of patients treated in a multidisciplinary TED clinic. METHODS: A medical record review of all patients who attended a TED clinic was performed. Data included demographics, medical history, laboratory tests, visual function tests, ocular examinations, clinical activity score (CAS), and assessment of quality-of-life (QOL). RESULTS: Clinic visits included 132 patients seen during 385 appointments at a TED clinic (mean 12 appointments per patient). Management of TED included medical treatments for 48 patients (36.3%) and surgical treatment for 56 (42.4%). There was a positive significant correlation between the CAS and thyroid-stimulating immunoglobulin (TSI) activity at the first visit and at the last follow-up visit (P < 0.01 and P < 0.02, respectively). However, no correlation was found between the CAS and the thyroid-stimulating hormone levels or between the free triiodothyronine (fT3) and fT4 levels at the first or last visit. There was a significant negative correlation between the CAS and color vision (-0.347, P < 0.01, Pearson correlation) at the first visit, but not between the CAS and visual acuity and visual field at either the first or last visit. Changes in the QOL and the CAS scores were significantly negatively correlated (-0.240, P < 0.01). CONCLUSIONS: Treatment and management decisions for TED should be based on multiple parameters including clinical examinations by ophthalmologists and endocrinologists, laboratory tests, and CAS and QOL scores.

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.

Clinical profile of 80-year-old and older thyroid eye disease patients.

PURPOSE: Thyroid eye disease (TED), an autoimmune orbital disorder, follows a time-of-onset bimodal peak: 40-44 and 60-64 years for women, 45-49 and 65-69 years for men. TED, however, can also commence in old age. The study's purpose was to evaluate TED in octo- and nonagenarians. METHODS: Medical records of 19 ≥ 80 years geriatric patients at time of diagnosis were compared to 122 TED patients, aged 20-79. A second analysis was performed after subdividing the control group into two age groups, ≤ 40 ("young group," 16 patients) and 41-79 years ("middle-aged group," 106 patients). RESULTS: The geriatric group's mean age was 84 years (80-94), 11 males and 8 females. Mean follow-up time was 16 months. Compared to the controls, the geriatric patients smoked less (p = 0.012), were more often hypothyroid (p = 0.019), and had concurrent myasthenia gravis (p = 0.02) at time of diagnosis. Diplopia was the most common presenting symptom among the elderly (p = 0.005) and proptosis among the controls, specifically the young group (p = 0.027). Bilateral signs were more common among seniors (p = 0.049). Optic neuropathy was diagnosed in 10% of the geriatric group (2/19) and 11% of middle-aged group (12/106), all being resolved after steroids or orbital decompression. Active disease (clinical activity score (CAS) score = > 3) was more common among the middle-aged group (p = 0.024) while the geriatric patients tended towards higher TED severity grades. Orbital decompression and eyelid repositioning surgeries were more common among the middle-aged group. Strabismus surgeries were more common among seniors. CONCLUSIONS: TED among octo- and nonagenarians has unique patterns, with different demographic features, more exposed to diplopia, hypothyroidism, association with myasthenia gravis, and bilateral involvement. Special attention should be given when medically managing this subgroup.

Neuro-Ophthalmic Phenotype of OPA3.

BACKGROUND: Type III 3-methylglutaconic aciduria (OPA 3) is a neuro-ophthalmologic syndrome consisting of early-onset bilateral optic atrophy. Since Costeff described the phenotype of 19 patients in 1989, several reports described approximately 50 patients, but most of them lack details about neuro-ophthalmic phenotype. Our aim was to characterize the clinical neuro-ophthalmic phenotype of this syndrome. METHODS: Nine patients underwent meticulous visual function history and medical documents' review. Results of best-corrected visual acuity (VA), color vision, visual field (VF), ocular motility, pupillary reaction, slit-lamp, and dilated fundus examinations were recorded. Optical coherence tomography (OCT) was performed whenever possible. RESULTS: The average VA was 1.4 ± 0.8 logarithm of the minimum angle of resolution. Poor vision was the presenting symptom in 5 patients. Six patients had decreased VA and variable degrees of optic atrophy. Humphrey VF testing of 7 patients revealed generalized depression in 5 and a cecocentral defect in 2. All patients demonstrated dysmetric saccades. Four patients had strabismus, 3 with exotropia, and one with esotropia. Seven patients had nystagmus. Ocular motility abnormality is possibly the result of cerebellar atrophy that was found in MRI studies of our patients. OCT of the retina was possible in 6 patients and revealed retinal nerve fiber layer (RNFL) thinning as well as average retinal thinning. Three patients, in whom ganglion cell layer-inner plexiform layer (IPL) measurement was possible, also showed diffused thinning. CONCLUSIONS: This study compiled data regarding neuro-ophthalmic manifestation of OPA 3 Type III patients. Contrary to established literature, poor vision was the presenting symptom in only 50% of our patients. This is the first report of OCT findings in 3MGA patients. The results demonstrated diffused thinning of the RNFL and ganglion cell complex-IPL with correlation to VA, which is in contrast to OPA1 patients in whom the most severe thinning is at the level of the papillomacular bundle. Average retinal thinning was identified at second and third decades of life, possibly resulting from early ganglion cell loss. These results may contribute to visual prognosis, and OCT may help monitor experimental therapies.

Cognitive Performance of Patients With Multiple Sclerosis and Optic Neuritis at Presentation.

BACKGROUND: Cognitive dysfunction is common among patients with multiple sclerosis (MS), but the effect of coexisting optic neuritis (ON) at the first presentation of multiple sclerosis on the course of cognitive decline is unknown. The purpose of this study was to assess whether ON at presentation has any effect on the progression of cognitive decline in MS. METHODS: Historical cohort study. We retrospectively compared the cognitive performance of patients with relapsing-remitting MS with and without ON at the time of MS diagnosis. Subjects were included if cognitive test results were available both at baseline and after at least 36 months from presentation and grouped based on the presence (MS-ON) or absence (MS-non-ON) of optic neuritis at presentation. RESULTS: One hundred seventy consecutive subjects with MS were found suitable, with a 1:2 male:female ratio and a mean age at diagnosis of 33.0 ± 10.9 years. Forty-six patients (27.1%) presented with ON. No significant differences were found in cognitive performance at onset between the 2 groups. Both groups had a similar follow-up duration. The prevalence of cognitive decline in the general score was significantly higher in the MS-ON group compared with the MS-non-ON group (6.5% vs 0%, respectively; P < 0.001), as well as in the attention (8.7% vs 1.6%; P = 0.046) and the executive function (17.4% vs 2.4%; P = 0.001) domains. CONCLUSIONS: Optic neuritis at presentation of MS is associated with a higher prevalence of cognitive decline over time. Potential benefit of early intervention to prevent cognitive decline may be warranted.

Ocular surface temperature differences in glaucoma.

PURPOSE: Accumulating evidence suggests that neuroinflammation and immune response are part of the sequence of pathological events leading to optic nerve damage in glaucoma. Changes in tissue temperature due to inflammation can be measured by thermographic imaging. We investigated the ocular surface temperature (OST) profile of glaucomatous eyes to better understand the pathophysiology of these conditions. METHODS: Subjects diagnosed with glaucoma (primary open angle glaucoma [POAG] or pseudo exfoliation glaucoma [PXFG]) treated at the Sam Rothberg Glaucoma Center (11/2019-11/2020.) were recruited. Healthy subjects with no ocular disease served as controls. The Therm-App thermal imaging camera was used for OST acquisition. Room and body temperatures were recorded, and the mean temperatures of the medial cantus, lateral cantus, and cornea were calculated with image processing software. RESULTS: Thermographic images were obtained from 52 subjects (52 eyes: 25 POAG and 27 PXFG) and 66 controls (66 eyes). Eyes with glaucoma had a significantly higher OST compared to controls (mean 0.9 ± 0.3°C, p < 0.005). The difference between the two groups remained significant after adjustment for age, sex, intraocular pressure (IOP) and room and body temperatures. Lens status and topical IOP-lowering medication did not significantly affect OST. A subgroup analysis revealed that the OST was higher among eyes with POAG compared to eyes with PXFG, but not significantly. CONCLUSIONS: Differences in the OST between glaucomatous and normal eyes strengthens current thinking that inflammation affects the pathophysiology of glaucoma. Longitudinal studies are warranted to establish the prognostic value of thermographic evaluations in these patients.

OCULAR SURFACE TEMPERATURE DIFFERENCES IN RETINAL VASCULAR DISEASES.

PURPOSE: To define the effect of age-related macular degeneration (AMD) and diabetic retinopathy (DR) on the ocular thermographic profile. METHODS: This retrospective cross-sectional study included subjects diagnosed with DR or AMD between January and April 2019. Individuals without ocular disease served as controls. A thermal imaging camera was used for ocular surface temperature (OST) acquisition. The mean temperatures of the medial cantus, lateral cantus, and cornea were calculated. RESULTS: Thermographic images were obtained from 133 subjects (260 eyes, 97 DR and 163 AMD) and 48 controls (55 eyes). Ocular surface temperature was higher among patients with AMD and lowest among patients with DR (P < 0.001). A subgroup analysis revealed that eyes with diabetic macular edema had significantly higher OSTs than DR eyes without diabetic macular edema. Moreover, the OST in eyes with diabetic macular edema was similar to the measurements of the AMD group. There were no differences in OSTs between neovascular and nonneovascular AMD eyes. CONCLUSION: Although AMD and DR are considered posterior segment conditions, their effect on OST implies that the entire globe is involved. Although both conditions result from similar multifactorial pathophysiologic changes, the differences in OST between DR and AMD might be due to dissimilarity in the balance of pathologic processes involved in each condition. Further research is required to better understand the pathophysiology of these diseases and their effect on OST as well as to determine the effect of vasculature, circulation, and tissue metabolism on ocular temperature.

Broadening the phenotype of LRRK1 mutations - Features of malignant osteopetrosis and optic nerve atrophy with intrafamilial variable expressivity.

Osteosclerotic metaphyseal dysplasia is a rare disorder which features osteosclerosis involving long bones, vertebrae, ribs, clavicles and the iliac crests. Additional features which have variably been reported include developmental delay, short stature, hypotonia and seizures. The disease is caused by pathogenic variants in the LRRK1 gene, and inherited in an autosomal recessive manner. We report three siblings (ages 14 years, 11.5 years and 0.9 years), born to consanguineous parents of Arab-Muslim descent, harboring a homozygous pathogenic variant in the LRRK1 gene (Chr15:101068759 AGGGGCT>A, c.5965_5970del TGGGGC, p.Trp1989Gly1990del). The patients displayed variable degrees of skeletal dysplasia, with the oldest sibling most severely affected, and the youngest infant with minor skeletal involvement. Two of the siblings exhibited normal neurological development, while the youngest sibling exhibited global developmental delay. None of the siblings had seizures; however, two of them exhibited nystagmus. Optic nerve involvement has not previously been reported to be part of the clinical spectrum of this disease. The degree of optic nerve involvement did not correlate with the degree of skeletal involvement. This indicates both intra-familial variable expressivity along with a broadening of the spectrum of LRRK1-associated disease. These findings warrant reconsideration of therapeutic strategies, including the possibility of hematopoietic stem cell transplantation (HSCT) as is performed in cases of malignant and intermediate forms of osteopetrosis.

Neuro-ophthalmologic outcomes of standard versus hypo-fractionated stereotactic radiotherapy of AVPM.

BACKGROUND: Most anterior visual pathway meningiomas (AVPM) are benign and slow-growing, but these tumors may affect visual functions, including visual acuity (VA) and visual field (VF). Due to location, most are treated non-surgically by fractionated stereotactic radiotherapy (FSRT), aiming to prevent tumor progression and visual functions deterioration. Unfortunately, FSRT in itself may affect visual functions. The current preferred treatment regimen (in terms of safety and effectiveness) is undetermined. While most cases are treated with conventional fractionation (cFSRT)-50.4-54 Gy in 28-30 fractions of 1.8-2 Gy, advances in technology have allowed shortening of total treatment length to hypofractionation (hSRT)-25-27 Gy in 3-5 fractions of 5-9 Gy. Our aim was to evaluate the association of radiotherapy regimen for treating AVPM (cFSRT vs. hSRT) with visual function outcomes (VA, VF) at the last neuro-ophthalmologic evaluation. METHODS: We conducted a retrospective cohort study of AVPM cases treated at Sheba Medical Center during 2004-2015. We compared cFSRT and hSRT regimens regarding visual function (VA, VF) outcomes at the last neuro-ophthalmologic evaluation. VA was determined by the logarithm of the minimum angle of resolution (LogMAR). VF was determined by the mean deviation (MD). A clinically relevant change in VA was defined as 0.2 LogMAR. RESULTS: 48 patients (13 receiving hSRT, 35 receiving cFSRT) were included, with a median follow-up of 55 months. No significant difference was evident regarding LogMAR or MD of involved eyes at the last evaluation. Six (17%) patients in the cFSRT group experienced clinically relevant VA deterioration in the involved eye, compared with six (46%) in hSRT (p = 0.06). CONCLUSION: Our findings, using comprehensive and meticulous investigation of visual outcomes, suggest that hSRT may be associated with higher risk for VA and VF deterioration in AVPM especially in ONSM. We recommend the use of cFSRT for ONSM.

Er:YAG laser-assisted filtration surgery: initial results in rabbits.

BACKGROUND: Glaucoma is a leading cause of global blindness, especially preventable blindness. The increased prevalence of glaucoma has led to a growing demand for newer, safer, more rapid, and simpler treatments for the reduction of intraocular pressure (IOP). In this study, we evaluated the safety and feasibility of performing filtration glaucoma surgery with an Ab-Interno Er:YAG laser in rabbits. METHODS: Nine New Zealand White rabbits age 16 weeks were studied. After subconjunctival injection of mitomycin C (MMC), a novel Ab-Interno Er:YAG laser probe was inserted into the anterior chamber (AC) through a clear corneal 1 mm paracentesis and directed at the trabecular meshwork adjacent to the MMC injection area. A pulsed laser beam was applied to create a patent sclerostomy connecting the AC to the subconjuctival space, resulting in a filtering bleb. The laser system used was the Er:YAG laser system - LAS25-FCU, (Pantec Biosolutions AG, Liechtenstein). Parameters used: Wave lengh: 2940 nm, Pulse length: 100-400 µsec,frequency: 250 Hz. Average laser power in accordance to the fiber tip diameter: 0.85 W(range 0.8-0.92 W). Complete ocular exams, including IOP measurements, were performed on 1, 7, 14, and 23 days postoperatively. Three rabbits were sacrificed on days 1, 14, and 23, and histological examinations were performed on all nine eyes. RESULTS: All procedures resulted in a functional medium-large superior bleb without significant complications. The bleb was sustained in all rabbits by day 14 and in one of the three rabbits that reached the last follow-up at 23 days. No cases of postoperative hypotony were observed. There was a transient significant reduction in mean IOP on postoperative days 5 and 7 (P = 0.028). Histopathological analysis revealed a patent full-thickness scleral tunnel with only a minor degree of surrounding coagulation necrosis. CONCLUSIONS: The Ab-Interno laser sclerostomy procedure is potentially safe and effective based on initial experience in an in-vivo rabbit animal model.

Young Adults With Anterior Ischemic Optic Neuropathy: A Multicenter Optic Disc Drusen Study.

PURPOSE: Optic disc drusen (ODD), present in 2% of the general population, have occasionally been reported in patients with nonarteritic anterior ischemic optic neuropathy (NA-AION). The purpose of this study was to examine the prevalence of ODD in young patients with NA-AION. DESIGN: Retrospective, cross-sectional multicenter study. METHODS: All patients with NA-AION 50 years old or younger, seen in neuro-ophthalmology clinics of the international ODDS (Optic Disc Drusen Studies) Consortium between April 1, 2017, and March 31, 2019, were identified. Patients were included if ODD were diagnosed by any method, or if ODD were excluded by enhanced-depth imaging optical coherence tomography (EDI-OCT) using ODDS Consortium guidelines. NA-AION eyes with ODD were termed "ODD-AION"; those without were termed "NODD-AION". RESULTS: A total of 65 patients (127 eyes) with NA-AION were included (mean 41 years old). Of the 74 eyes with NA-AION, 51% had ODD-AION, whereas 43% of fellow eyes without NA-AION had ODD (P = .36). No significant differences were found between ODD-AION and NODD-AION eyes in terms of Snellen best-corrected VA or perimetric mean deviation. According to EDI-OCT results, 28% of eyes with NODD-AION had peripapillary hyperreflective ovoid mass-like structures (PHOMS); 7% had hyperreflective lines, whereas 54% with ODD-AION had PHOMS; and 66% had hyperreflective lines (P = .006 and P < .001, respectively). CONCLUSIONS: Most of these young NA-AION patients had ODD. This indicates that ODD may be an independent risk factor for the development of NA-AION, at least in younger patients. This study suggests ODD-AION be recognized as a novel diagnosis.

Prognostic value of JAK2V617F mutation in pseudotumor cerebri associated with cerebral venous sinus thrombosis.

OBJECTIVES: To examine the clinical characteristics and prognosis of cerebral venous sinus thrombosis (CVST) that presented as pseudotumor cerebri (PTC) patients with JAK2V617F mutation. METHODS: Medical records of all consecutive patients that presented with PTC and a JAK2V617F mutation who were treated were retrospectively reviewed. Data regarding demographics and ocular presenting symptoms and signs, neurological signs, hematological factors treatment, and prognosis were collected. RESULTS: The most common presenting symptoms were headache (5 patients, 83.3%) and visual obscurations (5 patients, 83.3%). CVST of the sagittal sinus and sigmoid sinus were the most common site of thrombus. Platelet count and hemoglobin count were higher than normal during follow-up. There was significant change in the disk edema degree as well as decline in retinal nerve fiber layer (RNFL) thickness (P < 0.001, P < 0.001, Matched pairs). There was no significant change in visual acuity (VA) or mean deviation (MD) during follow-up (P = 0.95, 0.64, respectively, Matched pairs). CONCLUSIONS: Pseudotumor cerebri resulting from CSVT in our patients with JAK2V617F mutation was frequent in young patients and needed medical and surgical treatment, without improvement in visual functions and in third caused poor visual outcome. Therefore, we believe that a screening test for JAK2V617F mutation should be considered for patients with CVST without known risk factor presenting with PTC, especially when sagittal sinus or sigmoid sinus involvement or thrombocytosis or high hemoglobin are found upon presentation. This might lead to more aggressive management which may improve the visual prognosis of those young patients.

Association of orbital decompression and the characteristics of subsequent strabismus surgery in thyroid eye disease.

OBJECTIVE: To examine the association of orbital decompression and the characteristics and outcome of subsequent strabismus surgery in patients with thyroid eye disease (TED). METHODS: Data on patients with TED who underwent orbital decompression at the Goldschleger Eye Institute, Sheba Medical Center, Israel, between January 1990 to December 2011 were extracted. The characteristics of decompression and strabismus surgeries were recorded. The outcomes and association of both surgical procedures were analyzed. Statistical analysis included distribution, Pearson correlation, and matched paired tests. RESULTS: 145 eyes underwent orbital decompression, of which 45 eyes (31.0%) underwent strabismus surgery. Esotropia developed in 70% of the patients. Men and smokers underwent strabismus surgeries after decompression procedures more frequently than women and nonsmokers (χ2 test, p = 0.07, 0.002). Moreover, patients who complained of diplopia before the decompression surgery underwent strabismus surgery more frequently (χ2 test, p = 0.005). Seventy-seven percent of the patients who underwent medial wall decompression developed esotropia (χ2 test, p = 0.004). CONCLUSIONS: To the best of our knowledge, this is the largest series in the literature examining the association between decompression and strabismus surgeries. The patients' characteristics and the orbital walls involved in the decompression procedures are associated with the characteristics of subsequent strabismus that develops thereafter. These findings may have significant implications in planning TED management.