Summary of Utah Project on Exfoliation Syndrome (UPEXS): using a large database to identify systemic comorbidities.

The purpose of the Utah Project on Exfoliation Syndrome (UPEXS) is to identify associations between exfoliation syndrome (XFS) and other diseases that share the commonality of abnormalities in elastin and Lysyl Oxidase-Like 1 gene regulation. The UPEXS is unique because it uses the Utah Population Database, which is linked to the Utah genealogy, that contains a compilation of large pedigrees of most families in the state of Utah that go back multiple generations (3 to ≥11). The health and medical records of these family members are linked to vital records and can be used effectively in studies focused on genetic disorders like XFS, where familial clustering of a disorder is a trend. There is increasing evidence that patients with XFS have a higher risk of certain systemic disorders that reflect the systemic tissue abnormalities of XFS. Epidemiological studies focused on patients with XFS have shown that there is an increased risk of these individuals developing other pathologies that have abnormalities in extracellular matrix metabolism and repair. UPEXS has focused on suspected comorbidities that involve abnormalities in elastin maintenance, a protein that plays a role in the makeup of the extracellular matrix. In this paper, the results from the analysis of chronic obstructive pulmonary disease, inguinal hernias, pelvic organ prolapse, obstructive sleep apnoea and atrial fibrillation are summarised along with the utility of using such a large dataset.

Association between Obstructive Sleep Apnea and Exfoliation Syndrome: The Utah Project on Exfoliation Syndrome.

PURPOSE: Exfoliation syndrome (XFS), the most common recognizable cause of open-angle glaucoma worldwide, is a systemic disorder with genetic predisposition due to variations in lysyl oxidase-like 1 (LOXL1) function, leading to altered elastin matrices in ocular and systemic tissues. Obstructive sleep apnea (OSA) is a highly prevalent disorder also involving elastic tissue dysfunction and is associated with glaucoma. Because of the similarities between the disorders, we sought to uncover any relationship in the prevalence of these diagnoses. DESIGN: Case-control, retrospective cohort study. PARTICIPANTS: A cohort of 81 735 patients diagnosed with OSA at ages 50 to 90 years was identified from medical records from 1996 to 2017 in the Utah Population Database. Case subjects were matched to random controls on sex and birth year in a 4:1 ratio. METHODS: International Classification of Diseases, Ninth Revision (ICD-9) codes or their Tenth Revision equivalent were used to define a diagnosis of OSA (ICD-9 327.23) and a diagnosis of XFS (ICD-9 365.52 and 366.11). Conditional logistic regression odds ratios (ORs) accounting for individual matching on sex and birth year were used to estimate the risk of XFS in patients with OSA. Models included adjustment for race, obesity, tobacco use, hypertension (HTN), atrial fibrillation (AF), and chronic obstructive pulmonary disease (COPD). MAIN OUTCOME MEASURE: Whether patients with OSA have an increased risk of diagnosis of XFS compared with controls without OSA. RESULTS: There was an increased risk of an XFS diagnosis in patients with OSA compared with non-OSA controls (OR, 1.27; 95% confidence interval [CI], 1.02-1.59; P = 0.03). In a stratification of patients by HTN diagnosis history, patients with OSA and HTN exhibited an increased risk of XFS compared with non-OSA controls with HTN (OR, 2.67; 95% CI, 2.06-3.46; P < 0.0001). CONCLUSIONS: Patients with OSA may be at an increased risk of XFS compared with patients without OSA, particularly in patients with a history of HTN.

Complicated case of Mycobacterium abscessus conjunctivitis in Sjögren's syndrome.

PURPOSE: To report a case of conjunctivitis due to Mycobacterium abscessus in the setting of keratoconjunctivitis sicca due to Sjögren's syndrome in the absence of other known risk factors such as surgery, trauma or immunosuppressive therapy. OBSERVATIONS: A 61-year-old woman with a history of keratoconjunctivitis sicca secondary to Sjögren's syndrome presented with dryness, irritation, redness, and discharge in her left eye for 2 months. She was diagnosed with chronic conjunctivitis and began a regimen of moxifloxacin and an ocular ointment of dexamethasone, neomycin, and polymyxin with no improvement of symptoms. Concurrent cultures grew Mycobacterium abscessus and the patient began treatment with amikacin drops, oral clarithromycin and intravenous imipenem, followed by amikacin drops, oral clarithromycin, and oral clofazimine, but her course was complicated by a perforated corneal ulcer that required a corneal patch graft. The patient eventually recovered despite persistent colonization. CONCLUSIONS/IMPORTANCE: We present a case of Mycobacterium abscessus conjunctivitis in a patient with keratoconjunctivitis sicca secondary to Sjögren's syndrome without previous history of surgery, trauma, or other known risk factors. Because of low suspicion and clinician awareness, ocular nontuberculous mycobacteria (NTM) infection may have a delayed diagnosis and treatment. Clinicians should consider NTM in the differential diagnosis in patients with autoimmune disease such as Sjögren's syndrome. Treatment may be lengthy, requiring topical and systemic antibiotics and is often complicated due to resistance.

Uveal and capsular biocompatibility of a new hydrophobic acrylic microincision intraocular lens.

PURPOSE: To evaluate uveal biocompatibility and capsular bag opacification of a new hydrophobic acrylic microincision intraocular lens (IOL) in comparison with a commercially available 1-piece hydrophobic acrylic IOL. SETTING: John A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. DESIGN: Experimental study. METHODS: Eight New Zealand rabbits underwent bilateral phacoemulsification and implantation of the preloaded Nanex multiSert IOL in one eye and a commercially available preloaded lens (AcrySof IQ in UltraSert, model AU00T0) in the contralateral eye. A slitlamp examination was performed weekly for 4 weeks. The rabbits were then killed humanely and their globes enucleated. Capsular bag opacification was assessed from the Miyake-Apple view, and the eyes were subjected to histopathologic evaluation. RESULTS: Postoperative inflammatory reactions were similar between the test and control eyes in the 8 New Zealand rabbits. The mean postmortem central posterior capsule opacification (PCO) was 0.93 ± 0.73 in the test group and 1.19 ± 0.53 in the control group. The mean postmortem peripheral PCO was 1.75 ± 0.92 in the test group and 2.06 ± 0.77 in the control group. Central and peripheral PCO scores were not statistically different between the test and control groups (P = .41 and P = .35, respectively, 2-tailed t test: paired 2-sample for means). CONCLUSIONS: A new 1-piece hydrophobic acrylic microincision IOL incorporating an ultraviolet-ozone treatment on the posterior surface performed similarly to a commercially available 1-piece hydrophobic acrylic IOL in terms of uveal and capsular biocompatibility in the rabbit model. To our knowledge, this is the first hydrophobic acrylic microincision IOL to demonstrate similar PCO performance when compared with a conventional, commercially available IOL.

Posterior capsule opacification prevention by an intraocular lens incorporating a micropatterned membrane on the posterior surface.

PURPOSE: To evaluate posterior capsule opacification (PCO) with a new hydrophobic acrylic intraocular lens (IOL) featuring a new micropatterned membrane, in comparison with a commercially available 1-piece hydrophobic acrylic IOL. SETTING: John A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. STUDY DESIGN: Experimental study. METHODS: Twelve New Zealand rabbits had bilateral phacoemulsification and implantation of a ClearSight unpatterned IOL (Group 1), a ClearSight Sharklet-patterned IOL (Group 2), or a control, commercially available IOL (Group 3) (8 IOLs in each group). Slit-lamp examination was performed weekly for 4 weeks. The rabbits were then killed humanely, and their globes enucleated. Capsular bag opacification was assessed from the Miyake-Apple view, and the eyes underwent histopathology. RESULTS: The mean postmortem central PCO was 1.87 ± 1.35 in Group 1, 1.06 ± 1.23 in Group 2, and 3.14 ± 0.89 in Group 3. Peripheral PCO was 2.18 ± 1.36 in Group 1, 1.5 ± 1.03 in Group 2, and 3.57 ± 0.53 in Group 3. When comparing central and peripheral PCO between Groups 1 and 3, the difference was not statistically significant, but it was statistically significant between Groups 2 and 3 (P = .003 and P = .0003, t test with Bonferroni correction). CONCLUSIONS: Unique discontinuous features comprising the micropattern allow for focal adhesions to be precisely guided and therefore controlling cell migration. The patterned membrane incorporated on the posterior surface of the IOL significantly reduced capsular bag opacification compared with a commercially available control IOL.

Using a Macro Lens for Anterior Segment Imaging in Rural Panama.

Background: Visual impairment, specifically anterior segment pathology, presents a significant burden of disease in the world. Introduction: Inexpensive tools are necessary to improve eye health of residents in developing countries where care is difficult to access. Our study aimed at determining whether a $5 macro lens attached to a smartphone camera is an effective anterior segment imaging method for screening diseases. Materials and Methods: Fifty four (n = 54) patients had anterior segment imaging performed by using an Easy Macro lens and an iPhone. Imaging was performed at the Floating Doctors' mobile clinic sites in Panama. Images were sent back and graded by two board-certified ophthalmologists using a modified version of the FOTO-ED scale. Statistical analysis was performed by using a Wilcoxon signed-rank test to compare grades between the two imaging modalities. Results: There was no significant difference in overall clinical utility of images obtained by the iPhone versus Easy Macro lens. The iPhone was significantly superior in imaging of the lens and conjunctiva, whereas the Easy Macro lens was superior in regards to the anterior chamber, iris, and lens. Discussion: The imaging modality that best captures pathology is dependent on what part of the anterior segment is being examined. An imaging protocol with a pair of images, one from a smartphone and one from a macro lens, would have significant clinical utility. Conclusion: Our study demonstrates how minimally trained users can deliver effective eye screening via a telemedicine-based approach in a resource-deprived setting. Future directions would be to develop a telemedicine protocol and determine whether it improves clinically measurable outcomes in patients.

Evaluation of the capsular safety of a new hybrid phacoemulsification tip in a cadaver eye model.

PURPOSE: To evaluate the use of a new hybrid phacoemulsification tip made with a high strength polymer overmold designed to prevent posterior capsule rupture (PCR) compared with a metal tip. SETTING: John A. Moran Eye Center, University of Utah, USA. DESIGN: Experimental study. METHODS: This ex vivo crossover study compared a new phacoemulsification tip to a metal tip. After a preliminary experiment applying the tips to the posterior capsule with the bevel down, 10 phakic cadaver eyes were prepared using the Miyake-Apple technique, after which each tip was tested in a series. After lensectomy, the posterior capsule was subjected to direct contact from the tip with the bevel up. The vacuum limit was set to 150 mm Hg, and the aspiration flow rate to 30 cc/min, after which the torsional power was increased by increments of 5% up to a maximum amplitude of 60%. The primary endpoint was the torsional power required to produce PCR. RESULTS: Vacuum alone did not cause PCR with either tip. All tests showed that a higher torsional power was required for PCR with the hybrid tip. With the bevel directed upward, the hybrid tip required higher torsional power before PCR than the metal tip (55% ± 10.0% [SD] versus 15% ± 4.1%, respectively) (P value < .001). This indicates that there is a lower likelihood for PCR with the hybrid tip. CONCLUSION: The hybrid tip used in this study can be an effective means of preventing surgical complications such as PCR. This tip would be useful for experienced surgeons as well as for those who are in training.

Surface deposits mimicking calcification on a hydrophobic acrylic intraocular lens.

We describe the case of a female patient who had 3-piece silicone intraocular lenses (IOLs) bilaterally implanted after uneventful surgeries in 2007. Nine years later, the IOLs were explanted and exchanged with 3-piece hydrophobic acrylic IOLs because of cloudiness on their posterior aspect. Two years later, a similar finding was observed on the hydrophobic acrylic IOLs, and it was more intense in the left eye. The IOL in this eye was explanted and submitted for microscopic evaluation, which demonstrated the presence of multiple deposits irregular in shape and size covering significant areas of anterior and posterior optic surfaces. Elemental analyses ruled out the presence of calcification. The surface deposits stained positive for proteins. To our knowledge, postoperative calcification has not been definitively associated with hydrophobic acrylic IOLs to date.

Long-Term Results of Femtosecond Laser-Enabled Keratoplasty With Zig-Zag Trephination.

PURPOSE: To report long-term visual and astigmatism outcomes in cases of zig-zag femtosecond laser-enabled penetrating keratoplasty (FLEK). METHODS: Retrospective review. Three hundred thirty-five eyes of 287 patients underwent (FLEK) with a zig-zag incision pattern. Patients were assessed preoperatively and underwent postoperative comprehensive examinations at standard intervals of 1, 3, 6, 9, and 12 months, and 6 months thereafter. Postoperative uncorrected distance visual acuity and spectacle-corrected distance visual acuity and manifest and topographical (Mrx cyl and Topo cyl) astigmatism were compared with preoperative values. RESULTS: Three hundred thirty-five eyes received FLEK with zig-zag configuration. Data are presented for the last recorded visit before any refractive procedure. Sutures were removed in 202 of 335 eyes at an average time to removal of 1.3 ± 1.1 years, and a mean follow-up period of 2.9 ± 2.1 years (range 0-10 years). After full suture removal, mean uncorrected distance visual acuity and spectacle-corrected distance visual acuity were logarithm of the minimum angle of resolution 0.84 (Snellen 20/138) ± 0.55 and 0.33 (Snellen 20/42) ± 0.33, respectively. Mean Mrx cyl and Topo cyl of these groups were 3.38 ± 2.22 and 4.77 ± 3.15, respectively. Of the total number of grafts, the rate of graft rejections was 14.0%, and the failure rate was 5.6%. CONCLUSIONS: The femtosecond laser-generated zig-zag-shaped incision results in lower manifest and topographical astigmatism than the reported average for conventional penetrating keratoplasty. Graft rejection and failure rates are similar to published data for conventional penetrating keratoplasty.

Visual acuity after cataract surgery in patients with optic neuropathies.

PURPOSE: To compare visual acuity outcomes of cataract surgery between patients with and without non-glaucomatous optic neuropathies. METHODS: Retrospective case-control study of patients with and without optic neuropathies who underwent cataract surgery from 2010 to 2017. For all eyes, the last preoperative best corrected visual acuity (BCVA) and first BCVA greater than 1 month postoperatively (to ensure sustained refractive stabilization) were recorded in logarithm of the minimum angle of resolution (logMAR) units. RESULTS: Thirty patients (42 eyes) with optic neuropathies and 30 control patients (42 eyes) underwent uncomplicated cataract surgery. The mean age at surgery was significantly younger in the optic neuropathy group (64 versus 71.2 years, p < 0.01). The mean improvement in visual acuity in the optic neuropathy group was 0.4 ± 0.6 logMAR units (roughly 4 Snellen lines) from 0.7 ± 0.8 units preoperatively to 0.3 ± 0.5 postoperatively. Between the cases and controls, there was no significant difference in preoperative BCVA (p = 0.48), postoperative BCVA (p = 0.42), or the mean improvement in BCVA (p = 0.82). When stratified by optic neuropathy subtype, patients with optic neuropathies secondary to multiple sclerosis (n = 12) or non-arteritic ischaemic optic neuropathy (n = 11) had significant improvement in BCVA postoperatively (p < 0.01 and p = 0.03, respectively). CONCLUSION: Contrary to what may be expected from such a severe ocular comorbidity, our data suggest that the mean BCVA improvement after cataract surgery in patients with non-glaucomatous optic neuropathies was comparable to that of control patients. Cataract surgery may be performed in patients with both optic neuropathies and advanced cataracts with a reasonable preoperative expectation that visual acuity improvement can be significant.

Penetrating Keratoplasty Using the Femtosecond Laser: A Comparison of Postoperative Visual Acuity and Astigmatism by Suture Pattern.

PURPOSE: To compare the effect of 3 common suturing techniques on eyes that received femtosecond laser-enabled penetrating keratoplasty (FLEK) with a zig-zag configuration at the Gavin Herbert Eye Institute, University of California- Irvine, California. METHODS: This is a retrospective chart review of a series of 319 eyes of 286 patients who underwent FLEK with a zig-zag configuration. One hundred fifty-seven eyes had running sutures, 136 eyes had simple interrupted sutures, and 26 eyes had combined sutures (single running and simple interrupted). The main outcome measures were uncorrected distance visual acuity, best spectacle-corrected visual acuity (BSCVA), and topographic astigmatism (TCyl). These parameters were recorded for the first year of follow-up regardless of suture removal status and also after full suture removal. RESULTS: At 3 months, the BSCVA of the running, interrupted, and combined suture groups was 0.22 (±0.2), 0.41 (±0.3), and 0.29 (±0.1), respectively (P < 0.01). The mean TCyl of the running, interrupted, and combined groups at 3 months was 3.95 (±2.7), 6.41 (±4.4), and 5.44 (±3.3) D, respectively (P < 0.01) All sutures were removed in 190 of 319 eyes, and at the last recorded visit, the mean BSCVA was 0.18 (±0.2), 0.34 (±0.2), and 0.19 (±0.2) logarithm of the minimum angle of resolution in the running, interrupted, and combined groups, respectively (P < 0.01) and mean TCyl was 4.51 (±2.8), 5.62 (±3.7), and 4.57 (±2.9) D, respectively (P = 0.11). CONCLUSIONS: Better visual acuity after full suture removal was observed in the running suture group; however, the subgroups of patients with keratoconus were similar after all sutures were removed.

Feasibility study of first-year medical students identifying cardiac anatomy using ultrasound in rural Panama.

BACKGROUND: There are over 15 million children who have cardiac anomalies around the world, resulting in a significant morbidity and mortality. Early recognition and treatment can improve the outcomes and lengthen life-expectancy of these patients. The NIH and WHO have promoted guidelines for screening for congenital cardiac anomalies using ultrasound in rural environments. METHODS: Our study took place in Bocas Del Toro, Panama where a mobile clinic was established for community healthcare screening and ultrasonographic evaluation by medical student volunteers and volunteer clinical faculty. This was a non-blinded, investigational study utilizing a convenience sample of pediatric patients presenting for voluntary evaluation. Seven first-year medical students were recruited for the study. These students underwent a training program for advanced cardiac ultrasound instruction, termed "Pediatric Echocardiography Cardiac Screening (PECS)". RESULTS: Ten patients were enrolled in the study. Nine patients had adequate images as defined by the PECS criteria and were all classified as normal cardiac pathology by the medical students, resulting in a sensitivity and specificity of 100%. A single patient was identified by medical students as having a pathologic pulmonic stenosis. This was confirmed as correct by a blinded ultrasonographer. CONCLUSIONS: In this pilot study, the first-year medical students were able to correctly identify pediatric cardiac anatomy and pathology in rural Panama after undergoing a 12-hour ultrasound PECS training session. We believe that with this knowledge, minimally trained practitioners can be used to screen for cardiac anomalies in rural Panama using ultrasound.

Feasibility study of first-year medical students identifying cardiac anatomy using ultrasound in rural Panama / 世界急诊医学杂志（英文）

BACKGROUND: There are over 15 million children who have cardiac anomalies around the world, resulting in a significant morbidity and mortality. Early recognition and treatment can improve the outcomes and lengthen life-expectancy of these patients. The NIH and WHO have promoted guidelines for screening for congenital cardiac anomalies using ultrasound in rural environments. METHODS: Our study took place in Bocas Del Toro, Panama where a mobile clinic was established for community healthcare screening and ultrasonographic evaluation by medical student volunteers and volunteer clinical faculty. This was a non-blinded, investigational study utilizing a convenience sample of pediatric patients presenting for voluntary evaluation. Seven first-year medical students were recruited for the study. These students underwent a training program for advanced cardiac ultrasound instruction, termed "Pediatric Echocardiography Cardiac Screening (PECS)". RESULTS: Ten patients were enrolled in the study. Nine patients had adequate images as defined by the PECS criteria and were all classified as normal cardiac pathology by the medical students, resulting in a sensitivity and specificity of 100％. A single patient was identified by medical students as having a pathologic pulmonic stenosis. This was confirmed as correct by a blinded ultrasonographer. CONCLUSIONS: In this pilot study, the first-year medical students were able to correctly identify pediatric cardiac anatomy and pathology in rural Panama after undergoing a 12-hour ultrasound PECS training session. We believe that with this knowledge, minimally trained practitioners can be used to screen for cardiac anomalies in rural Panama using ultrasound.