ARHGEF-10 gene mutation presenting as orbital inflammatory syndrome.

Rho guanine nucleotide exchange factor 10 (ARHGEF-10) is a RHO GTPase that has a role for neural morphogenesis, however its effect on the eyes remains unknown. Here, we report a 44-year-old man who presented with eyelid swelling along with a history of bilateral hand contractures, high-arched feet and muscle wasting, who was found to have an ARHGEF-10 mutation. Neuroimaging was significant for numerous nerve-based cystic abnormalities in the bilateral orbits and throughout the neuraxis, and an orbital biopsy revealed S-100 and SOX-10 positive lesion consistent with pseudocysts. While the role of ARHGEF-10 remains unclear, further research is warranted to further describe its clinical manifestations.

Adult-onset asthma and periocular xanthogranuloma - A rare infiltrative disease of the orbit and eyelid.

PURPOSE: To present a case of adult onset asthma with periocular xanthogranuloma (AAPOX), and discuss existing literature on adult orbital xanthogranulomatous diseases (AOXGDs) and their treatment. OBSERVATIONS: A 63 year old male presented with progressive bilateral eyelid swelling with overlying yellow plaques associated with asthma. CT scan showed periorbital swelling with enlargement of the superior and lateral rectus muscles bilaterally. Biopsy demonstrated orbital xanthogranulomatous disease with increased IgG4 plasma cells. The patient was treated with intralesional triamcinolone, oral prednisone, and cyclophosphamide without significant improvement. Surgical debulking was eventually performed which improved his external symptoms until he was lost to follow up 15 months later. CONCLUSIONS AND IMPORTANCE: AOXGDs are a group of rare infiltrative diseases of the eyelids and orbit that can be associated with significant systemic morbidities. While they all have similar underlying histopathologic features, appreciating the clinical difference between these diseases is important in understanding patient prognosis and ensuring appropriate clinical monitoring. There is also growing research demonstrating that AAPOX, along with other AOXGDs, may represent part of a continuum of IgG4 related disease, similar to what is seen in this case. There is currently no reliably effective treatment for AOXGDs, and additional research into the management of these diseases is necessary.

Anterior uveitis secondary to intraocular fiber from a hair enhancement product.

PURPOSE: To report a case of anterior uveitis caused by migration of cosmetic "hair-building fibers" into the anterior chamber and discuss previous literature describing ocular disease caused by small foreign fibers. OBSERVATIONS: A 73 year old male presented with his first episode of acute anterior uveitis. He was found to have a white fiber penetrating through the cornea into the anterior chamber. This foreign body was removed. The patient identified the material as a being from the cosmetic hair-building fiber product he had been using called Toppik. He was treated with topical steroids and antibiotic drops. The uveitis resolved without recurrence by six weeks after his initial presentation. CONCLUSIONS: Small synthetic and non-synthetic fibers represent sources of both superficial and intraocular injury. Careful examination of the anterior chamber is critical in patients with new acute uveitis to identify possible foreign fibers that can migrate through the cornea. While medical therapy with topical steroids may suffice for treatment in many cases, intraocular persistence of these materials can result in recurrent inflammation, ocular hypertension, or further migration into the posterior chamber which may require surgical intervention.

Comparison of training effect on tremor using 2 training modules.

PURPOSE: To compare training effect of 2 training models-a surgical simulator anti-tremor module and a paper version-on tremor and time-to-task completion. SETTING: Ophthalmology Department, Veterans Affairs Boston Healthcare System, Jamaica Plain, Massachusetts, USA. DESIGN: Prospective crossover study. METHODS: Trainees completed simulator and paper training modules (baseline test, 3 training sessions, posttraining test, and final test) with their dominant and nondominant hands. The change in tremor, number of paper errors, and time-to-task completion in dominant and nondominant hands were compared. The 2 training modules were compared using nonparametric tests. RESULTS: The study comprised 19 trainees. There was a moderate correlation between average tremor values (simulator, 3-dimensional module) and paper errors (paper, 2-dimensional module) (Spearman ⍴ = 0.35, P < .0001). Practice on the simulator or paper modules did not reduce tremor significantly from baseline to final tasks for both hands combined (P = .12, simulator; P = .2, paper). Practice on the training modules improved time-to-task completion in the simulator module and paper module (both P < .0001). The improvement in time from baseline to final tasks was greater in the nondominant hands in the simulator module (improvement 64.5% over baseline time) than in the paper module (53.6% over baseline time). CONCLUSION: Practice might not reduce tremor but improved the outcome measure of time, and results suggest that trainees can learn to compensate for tremor in both hands, which is important in bimanual microsurgery.

Impact of First Eye versus Second Eye Cataract Surgery on Visual Function and Quality of Life.

PURPOSE: To compare the impact of first eye versus second eye cataract surgery on visual function and quality of life. DESIGN: Cohort study. PARTICIPANTS: A total of 328 patients undergoing separate first eye and second eye phacoemulsification cataract surgeries at 5 veterans affairs centers in the United States. Patients with previous ocular surgery, postoperative endophthalmitis, postoperative retinal detachment, reoperation within 30 days, dementia, anxiety disorder, hearing difficulty, or history of drug abuse were excluded. METHODS: Patients received complete preoperative and postoperative ophthalmic examinations for first eye and second eye cataract surgeries. Best-corrected visual acuity (BCVA) was measured 30 to 90 days preoperatively and postoperatively. Patients completed the National Eye Institute Visual Functioning Questionnaire (NEI-VFQ) 30 to 90 days preoperatively and postoperatively. The NEI-VFQ scores were calculated using a traditional subscale scoring algorithm and a Rasch-refined approach producing visual function and socioemotional subscale scores. MAIN OUTCOME MEASURES: Postoperative NEI-VFQ scores and improvement in NEI-VFQ scores comparing first eye versus second eye cataract surgery. RESULTS: Mean age was 70.4 years (±9.6 standard deviation [SD]). Compared with second eyes, first eyes had worse mean preoperative BCVA (0.55 vs. 0.36 logarithm of the minimum angle of resolution (logMAR), P < 0.001), greater mean BCVA improvement after surgery (-0.50 vs. -0.32 logMAR, P < 0.001), and slightly worse postoperative BCVA (0.06 vs. 0.03 logMAR, P = 0.039). Compared with first eye surgery, second eye surgery resulted in higher postoperative NEI-VFQ scores for nearly all traditional subscales (P < 0.001), visual function subscale (-3.85 vs. -2.91 logits, P < 0.001), and socioemotional subscale (-2.63 vs. -2.10 logits, P < 0.001). First eye surgery improved visual function scores more than second eye surgery (-2.99 vs. -2.67 logits, P = 0.021), but both first and second eye surgeries resulted in similar improvements in socioemotional scores (-1.62 vs. -1.51 logits, P = 0.255). CONCLUSIONS: Second eye cataract surgery improves visual function and quality of life well beyond levels achieved after first eye cataract surgery alone. For certain socioemotional aspects of quality of life, second eye cataract surgery results in comparable improvement to first eye cataract surgery.

Outcomes after cataract surgery in eyes with pseudoexfoliation: Results from the Veterans Affairs Ophthalmic Surgery Outcomes Data Project.

OBJECTIVE: To compare clinical outcomes of cataract surgery in eyes with and without pseudoexfoliation (PXF). DESIGN: Retrospective deidentified data analysis. PARTICIPANTS: A total of 123 PXF and 4776 non-PXF eyes of patients who underwent cataract surgery. METHODS: We compared data on visual acuity, Visual Function Questionnaire (VFQ)-based quality of life, and complications in PXF and non-PXF eyes from the Veterans Affairs (VA) Ophthalmic Surgery Outcomes Data Project across 5 VA medical centres. RESULTS: Pupillary expansion devices were used in 31 (25.2%) PXF cases and 398 (8.4%) non-PXF cases (p < 0.0001). Capsular tension rings were used in 6 (4.9%) PXF cases and 55 (1.2%) non-PXF cases (p < 0.004). The following complications occurred more frequently in PXF cases: zonular dehiscence without vitrectomy (4 [3.3%] PXF cases vs 40 [0.8%] non-PXF cases p = 0.02), persistent inflammation (28 [24.1%] vs 668 [14.5%]; p = 0.007), and persistent intraocular pressure elevation (5 [4.3%] vs 68 [1.5%]; p = 0.03). Best corrected visual acuity (BCVA) improved in both groups after 1 month, but 87 (83.7%) PXF cases achieved postoperative BCVA better than or equal to 20/40 compared to 3991 (93.8%) non-PXF cases (p = 0.0003). There was no significant difference in the postoperative composite VFQ scores between PXF (82.1 ± 16.9) and non-PXF cases (84.2 ± 16.8, p = 0.09). CONCLUSIONS: Several complications occurred more frequently in the PXF group compared to the non-PXF group, and fewer PXF cases achieved BCVA better than or equal to 20/40. Despite this, both groups experienced similar improvement in vision-related quality of life after cataract surgery.

CONSTRUCT AND FACE VALIDITY OF THE EYESI INDIRECT OPHTHALMOSCOPE SIMULATOR.

PURPOSE: To evaluate construct and face validity of the Eyesi Binocular Indirect Ophthalmoscope Simulator. METHODS: The performance of 25 medical students (Group A) was compared with that of 17 ophthalmology and optometry trainees (Group B) on the Eyesi Binocular Indirect Ophthalmoscope Simulator. During the course of a single session, each participant viewed an orientation module followed by an instruction session and a demonstration case, and performed 6 cases of progressively increasing difficulty (4 levels) and a 10-question face validity questionnaire. Outcomes included total score, total examination time, percent retina examined, and duration of eye exposure to light. RESULTS: Group B achieved significantly better total scores than Group A on all difficulty levels (P = 0.02, P = 0.001, P = 0.001, and P = 0.0001, for Levels 1-4, respectively) and had a significantly faster mean duration of examination (8 minutes 58 seconds vs. 5 minutes 21 seconds, P < 0.0001). Medical students reported higher scores in the face validity questionnaire for the simulator experience being helpful at orienting them to true indirect ophthalmology, and that further training on the simulator would improve their skills in the clinic (P = 0.03 for all). CONCLUSION: The Eyesi Binocular Indirect Ophthalmoscope Simulator has significant construct and face validity and shows promise for medical education.

A rare case of Phialemonium obovatum keratitis.

PURPOSE: We report a rare case of Phialemonium obovatum fungal keratitis in a patient sustaining a corneal laceration from impact with a piece of moldy plaster. OBSERVATIONS: The patient was treated with topical voriconazole with resolution of active keratitis and formation of a stromal scar. The patient's final visual acuity was 20/20 in the affected eye. CONCLUSIONS AND IMPORTANCE: There is only one other case of Phialemonium obovatum keratitis reported in the literature. The patient in the previous case required amniotic membrane transplantation for persistent stromal melting with resultant visual acuity of hand motions only. The present case demonstrates that the early use of topical antifungal medication with close follow-up can prevent corneal perforation and result in excellent visual acuity.

Management of Corneal Bee Sting Injuries.

PURPOSE: To review the management of keratitis after corneal bee stings and to report a case of deep stromal corneal infiltrate secondary to a retained bee stinger managed conservatively in a patient who presented three days after unsanitary manipulation of the stinger apparatus. METHODS: Case report and review of literature. RESULTS: A 57-year-old male beekeeper was evaluated for pain, blurry vision, and photosensitivity after a corneal bee sting. Of note, the venom sac had been removed with dirty tweezers three days prior to his visit. On exam, a focal infiltrate with diffuse edema was seen surrounding a retained bee stinger in the peripheral cornea. Trace cells in the anterior chamber were also noted. Based on a high suspicion for infectious keratitis, a conservative treatment strategy was elected. Administration of broad-spectrum topical antibiotics with concomitant abstention of corticosteroids led to rapid resolution of the symptoms. Over 16 months of follow-up, the stinger has remained in situ without migration and the patient has maintained 20/20 visual acuity without complications. There is debate on the preferred method for the management of corneal injury secondary to bee stings, especially when it is associated with a retained stinger. We herein present our findings in our appraisal of reported cases. CONCLUSION: In the aftermath of an ocular bee sting, close surveillance for inflammation and infection is essential. Individual manifestations of these injuries vary in timing, type, and severity; therefore, the accessibility of the stinger and the evolving clinical picture should guide therapeutic decisions.

American Society of Anesthesiologists classification in cataract surgery: Results from the Ophthalmic Surgery Outcomes Data Project.

PURPOSE: To explore the association of American Society of Anesthesiologists (ASA) classification with cataract surgery outcomes. SETTING: Five Veterans Affairs Medical Centers, United States. DESIGN: Retrospective observational cohort study. METHODS: The study analyzed the outcomes of cataract surgery cases. Corrected distance visual acuity (CDVA), unanticipated events, and vision-related quality of life (VRQL) were assessed using the National Eye Institute Visual Function Questionnaire (NEI-VFQ), comparing ASA classes I through IV. For some analyses, ASA classes I and II were designated as Group A and ASA classes III and IV were designated Group B. RESULTS: Of the 4923 cases, 875 (17.8%) were in Group A, 4032 (81.9%) were in Group B, and 16 (0.3%) had missing data. The mean CDVA and mean composite NEI-VFQ score improved after cataract surgery in both groups (P < .0001); however, Group A had a better mean postoperative CDVA and postoperative VFQ composite scores than Group B (P < .0001, both outcomes). A higher ASA class was associated with an increased risk for 2 unanticipated events; that is, clinically significant macular edema (CSME) (Group A: 4 [0.47%] versus Group B: 50 [1.28%]; adjusted odds ratio [OR], 3.02; 95% confidence interval [CI], 1.02-13.05; P = 0.04) and readmission to the hospital within 30 days (2 [0.23%] versus 56 [1.41%]; OR, 8.26; 95% CI, 1.71-148.62; P = .004) CONCLUSIONS: Among United States veterans, the ASA classification could be an important predictor of VRQL and visual outcomes. In this cohort, it was associated with an increased risk for 2 serious unanticipated events-CSME and readmission to the hospital-both costly, unwanted outcomes. FINANCIAL DISCLOSURE: Dr. Vollman is a consultant to Forsight Vision5. None of the authors has a financial or proprietary interest in any material or method mentioned.

Outcomes of cataract surgery with residents as primary surgeons in the Veterans Affairs Healthcare System.

PURPOSE: To explore visual outcomes, functional visual improvement, and events in resident-operated cataract surgery cases. SETTING: Veterans Affairs Ophthalmic Surgery Outcomes Database Project across 5 Veterans Affairs Medical Centers. DESIGN: Retrospective data analysis of deidentified data. METHODS: Cataract surgery cases with residents as primary surgeons were analyzed for logMAR corrected distance visual acuity (CDVA) and vision-related quality of life (VRQL) measured by the modified National Eye Institute Vision Function Questionnaire and 30 intraoperative and postoperative events. In some analyses, cases without events (Group A) were compared with cases with events (Group B). RESULTS: The study included 4221 cataract surgery cases. Preoperative to postoperative CDVA improved significantly in both groups (P < .0001), although the level of improvement was less in Group B (P = .03). A CDVA of 20/40 or better was achieved in 96.64% in Group A and 88.25% in Group B (P < .0001); however, Group B had a higher prevalence of preoperative ocular comorbidities (P < .0001). Cases with 1 or more events were associated with a higher likelihood of a postoperative CDVA worse than 20/40 (odds ratio, 3.82; 95% confidence interval, 2.92-5.05; P < .0001) than those who did not experience an event. Both groups had a significant increase in VRQL from preoperative levels (both P < .0001); however, the level of preoperative to postoperative VRQL improvement was significantly less in Group B (P < .0001). CONCLUSION: Resident-operated cases with and without events had an overall significant improvement in visual acuity and visual function compared with preoperatively, although this improvement was less marked in those that had an event. FINANCIAL DISCLOSURE: None of the authors has a financial or proprietary interest in any material or method mentioned.

Ophthalmic Surgical Simulation in Training Dexterity in Dominant and Nondominant Hands: Results From a Pilot Study.

PURPOSE: To determine whether a structured training program using the validated EYESI surgical simulator improves dexterity in nondominant (ND) hands. SETTING: Academic tertiary referral center. DESIGN: Nonrandomized, prospective study. METHODS: Subjects who chose to participate and provided informed consent completed a structured simulation training program, which included a baseline test, 3 sessions of repeated tasks, and a final test on capsulorhexis in dominant (D) and ND hands. Participants completed demographic and satisfaction questionnaires. Performances at each session were recorded. We compared overall scores at baseline and at the end of the study, and analyzed trends over time. Statistical analysis was performed using JMP by SAS. RESULTS: Overall, 14 subjects completed the training program. In all, 3 (21.4%) were attending physicians and 11 (78.6%) were trainees. There was a significant improvement in the average overall scores (baseline vs. final) in both the D hand (33.4 vs. 46.5; p < 0.05) and the ND hand (28.9 vs. 47.7; p < 0.001). The structured training program demonstrated significantly faster performance times in both hands at the end of the study (D p< 0.001, ND p < 0.02). However, the learning curve was significantly steeper in the ND hand (p < 0.01). Participants agreed that simulation training improved the ND hand dexterity. CONCLUSIONS: We found a significantly greater trend for improvement in the ND compared with the D hand. These results suggest that an elaborate, structured curriculum targeting teaching dexterity results in better simulated performance.

Effects of tear gases on the eye.

Chemical agents that target the eyes have been a popular choice for law enforcement during riots and for military training for nearly a century. The most commonly used agents are chloroacetophenone (formerly sold as Mace), o-chlorobenzylidene malononitrile, and oleoresin capsicum (OC or pepper spray, current ingredient for Mace). Initially, most severe ocular injuries were caused by the explosive force rather than the chemical itself. The development of sprays reduced the mechanical severity of ocular injuries, but resulted in a variety of chemical injuries. The effects on eyes include conjunctival injection, complete corneal epithelial defects, pseudopterygium, corneal neovascularization, persistent conjunctivalization, corneal opacities, and reduced visual acuity. Current management, based on limited human studies, emphasizes decontamination and symptomatic treatment. We review the literature related to clinical and histopathologic effects of tear gas agents on the eye and their management.

Cataract Surgery Outcomes in Glaucomatous Eyes: Results From the Veterans Affairs Ophthalmic Surgery Outcomes Data Project.

PURPOSE: To compare visual acuity outcomes, vision-related quality of life, and complications related to cataract surgery in eyes with and without glaucoma. DESIGN: Retrospective cohort study. METHODS: Cataract surgery outcomes in cases with and without glaucoma from the Veterans Affairs Ophthalmic Surgical Outcomes Data Project were compared. RESULTS: We identified 608 glaucoma cases and 4306 controls undergoing planned cataract surgery alone. After adjusting for age, pseudoexfoliation, small pupil, prior ocular surgery, and anterior chamber depth, we found that glaucoma cases were more likely to have posterior capsular tear with vitrectomy (odds ratio [OR] 1.8, P = .03) and sulcus intraocular lens placement (OR 1.65, P = .03) during cataract surgery. Glaucoma cases were more likely to have postoperative inflammation (OR 1.73, P < .0001), prolonged elevated intraocular pressure (OR 2.96, P = .0003), and additional surgery within 30 days (OR 1.92, P = .03). Mean best-corrected visual acuity (BCVA) and Visual Function Questionnaire (VFQ) scores significantly improved after cataract surgery in both groups (P < .0001), but there were larger improvements in BCVA (P = .01) and VFQ composite scores (P < .0001) in the nonglaucoma vs the glaucoma group. A total of 3621 nonglaucoma cases (94.1%) had postoperative BCVA 20/40 or better, compared to 466 glaucoma cases (89.6%) (P = .0003). CONCLUSIONS: Eyes with glaucoma are at increased risk for complications and have more modest visual outcomes after cataract surgery compared to eyes without glaucoma. Despite this, glaucoma patients still experience significant improvement in vision-related outcomes after cataract extraction. Further study is needed to explore potential factors that influence cataract surgery outcomes in glaucomatous eyes.

Functional Visual Improvement After Cataract Surgery in Eyes With Age-Related Macular Degeneration: Results of the Ophthalmic Surgical Outcomes Data Project.

PURPOSE: To determine if cataract surgery on eyes with AMD confers as much functional visual improvement as surgery on eyes without retinal pathology. METHODS: This is a retrospective analysis of 4924 cataract surgeries from the Veterans Healthcare Administration Ophthalmic Surgical Outcomes Data Project (OSOD). We included cases of eyes with AMD that had both preoperative and postoperative NEI-VFQ-25 questionnaires submitted and compared their outcomes with controls without retinal pathology. We excluded patients with other retinal pathologies (740 patients). The analyses compared changes in visual acuity and overall functional visual improvement and its subscales using t-tests, multivariate logistic regressions, and linear regression modeling. RESULTS: Preoperative and postoperative questionnaires were submitted by 58.3% of AMD and 63.8% of no retinal pathology cases (controls). Analysis of overall score showed that cataract surgery on eyes with AMD led to increased visual function (13.8 ± 2.4 NEI-VFQ units, P < 0.0001); however, increases were significantly less when compared with controls (-6.4 ± 2.9 NEI-VFQ units, P < 0.0001). Preoperative best-corrected visual acuity (preBCVA) in AMD was predictive of postoperative visual function (r = -0.38, P < 0.0001). In controls, postoperative visual function was only weakly associated with preBCVA (r = -0.075, P = 0.0002). Patients with AMD with vision of 20/40 or better had overall outcomes similar to controls (-2.2 ± 4.7 NEI-VFQ units, P = 0.37). CONCLUSIONS: Cataract surgery on eyes with AMD offers an increase in functional visual improvement; however, the amount of benefit is associated with the eye's preBCVA. For eyes with preBCVA of 20/40 or greater, the improvement is similar to that of patients without retinal pathology. However, if preBCVA is less than 20/40, the amount of improvement was shown to be significantly less and decreased with decreasing preBCVA.

Management of mydriasis and pain in cataract and intraocular lens surgery: review of current medications and future directions.

The maintenance of mydriasis and the control of postoperative pain and inflammation are critical to the safety and success of cataract and intraocular lens replacement surgery. Appropriate mydriasis is usually achieved by topical and/or intracameral administration of anticholinergic agents, sympathomimetic agents, or both, with the most commonly used being cyclopentolate, tropicamide, and phenylephrine. Ocular inflammation is common after cataract surgery. Topical steroids and nonsteroidal anti-inflammatory drugs are widely used because they have been proved effective to control postsurgical inflammation and decrease pain. Topical nonsteroidal anti-inflammatory drugs have also been shown to help maintain dilation. However, use of multiple preoperative drops for pupil dilation, inflammation, and pain control have been shown to be time consuming, resulting in delays to the operating room, and they cause dissatisfaction among perioperative personnel; their use can also be associated with systemic side effects. Therefore, ophthalmologists have been in search of new options to streamline this process. This article will review the current medications commonly used for intraoperative mydriasis, as well as pain and inflammation control. In addition, a new combination of ketorolac, an anti-inflammatory agent, and phenylephrine, a mydriatic agent has recently been designed to maintain intraoperative mydriasis and to reduce postoperative pain and irritation from intraocular lens replacement surgery. Two Phase III clinical trials evaluating this combination have demonstrated statistically significant differences when compared to placebo in maintaining intraoperative mydriasis (P<0.00001) and in reducing pain in the early postoperative period (P=0.0002). This medication may be of benefit for use in cataract and lens replacement surgery in the near future.