Faculty Bibliography

PURPOSE/OBJECTIVE:The purpose of this study was to describe the deep phenotype of congenital corneal opacities (CCO) in patients with 22q11.2 deletion syndrome (22q11.2 DS) and to identify putative regions or genes that could explain the CCO. METHODS:A retrospective chart review was conducted to identify patients with 22q11.2 DS seen in the ophthalmology clinic of a tertiary referral children's hospital. Thirty patients were identified, with molecular confirmation. Twenty-six did not show structural anterior segment anomalies aside from posterior embryotoxon (n = 4), whereas 4 had bilateral CCO, of which 3 had preoperative images. We reviewed medical, operative, and pathology reports; anterior segment optical coherence tomography; high-frequency ultrasound; histopathologic slides; and genetic testing. To identify putative genes responsible for CCO, chromosomal breakpoints in patients with and without CCO were compared. RESULTS:In the 3 patients with preoperative imaging and CCO, a pattern of paracentral corneal opacification with central clearing accompanied by iridocorneal or keratolenticular adhesions was observed. Anterior segment optical coherence tomography and histopathologic images showed central stromal thinning with a residual structure consistent with Descemet membrane. One patient presented at birth with unilateral corneal perforation, suggestive of likely stromal thinning. A comparison of the breakpoints across all cases failed to reveal unique regions or genes in patients with CCO. CONCLUSIONS:22q11.2 DS can rarely be associated with CCO. We describe a consistent pattern of central clearing related to posterior stromal thinning, with or without ICA/KLA. Possible candidate genes for corneal opacification in 22q11.2 DS remain elusive.

OBJECTIVE:To identify factors associated with receipt of endothelial keratoplasty (EK) and penetrating keratoplasty (PK) in patients with Fuchs' endothelial corneal dystrophy (FECD). DESIGN/METHODS:Retrospective cohort study. PARTICIPANTS/METHODS:Medicare beneficiaries ≥65 years with a diagnosis of FECD between 2011-2019. METHODS:The 100% Medicare fee-for-service administrative claims database was queried for treatment-naive patients with a diagnosis of FECD. A multivariable logistic model including age, race and ethnicity, sex, geography, ocular comorbidities and surgeries, Charlson comorbidity index (CCI), and socioeconomic status was used to identify factors associated with receipt of EK and PK. Kaplan Meier survival analyses were performed to determine the rate of EK after cataract surgery and complex/other anterior segment surgery. MAIN OUTCOME MEASURES/METHODS:The factors associated with receipt of a first-time EK or PK, plus the rate of EK following cataract or complex/other anterior segment surgery. RESULTS:Of 719066 beneficiaries identified, 31372 (4.4%) received a first-time EK, and 2426 (0.3%) received a first-time PK. In a multivariable analysis, female sex decreased likelihood of both PK and EK (for EK, aOR 0.83 [95% CI 0.81-0.85]; for PK, aOR 0.84 [95% CI 0.78-0.92]), while Western residence (1.33 [1.29-1.38]; 1.25 [1.11-1.42]) compared to Southern and history of complex/other anterior segment surgery (1.62 [1.54-1.70]; 5.52 [4.97-6.12]) increased likelihood of both procedures. Compared to White (non-Hispanic), likelihood of EK was decreased for Black (0.76 [0.72-0.80]), Asian/Pacific Islander (0.54 [0.48-0.61]), and Hispanic/Latino (0.62 [0.55-0.70]) race and ethnicity, while for the same groups likelihood of PK was increased (for Black 1.32 [1.14-1.53]; Asian/Pacific Islander 1.46 [1.13-1.89]; Hispanic/Latino 1.62 [1.25-2.11]). Additional factors decreasing likelihood of EK were increasing age (for >85, 0.60 [0.58-0.62]), Northeastern residence (0.81 [0.78-0.84]) compared to Southern, and Charlson comorbidity index score of 7+ (0.50 [0.44-0.57]) compared to 0. Following cataract or complex/other anterior segment surgery, rates of EK were 1.3% and 3.3% at one year, and 2.3% and 5.6% at eight years, respectively. CONCLUSIONS:In a multivariable analysis, female beneficiaries are less likely to receive EK or PK for FECD compared to males, whereas non-White beneficiaries are less likely to receive EK and more likely to receive PK compared to White beneficiaries.

ABSTRACT/UNASSIGNED:Contact lenses are a safe and effective method for correction of refractive error and worn by an estimated 45 million Americans. Because of the widespread availability and commercial popularity of contact lenses, it is not well appreciated by the public that contact lenses are U.S. Food and Drug Administration (FDA)-regulated medical devices. Contact lenses are marketed in numerous hard and soft materials that have been improved over decades, worn in daily or extended wear, and replaced in range of schedules from daily to yearly or longer. Lens materials and wear and care regimens have impact on the risks of contact lens-related corneal inflammatory events and microbial keratitis. This article reviews contact lens safety, with specific focus on the correction of refractive error in healthy eyes.

BACKGROUND:Despite females comprising 50.8% of the U.S. population, the percentage of females in the physician workforce is only 36.3%. Studies have examined sex trends within select specialties, however there is insufficient literature studying trends across all specialties. In this study, the authors examined trends in the proportion of female residents from 2011 to 2019 across all specialties, including both surgical and non-surgical.  METHODS: Data on the proportion of female residents from 2011 to 2019 in all specialties was extracted from the Accreditation Council for Graduate Medical Education (ACGME) Data Resource Books and analyzed with the chi-square test for trend. RESULTS:From 2011 to 2019, there was a statistically significant increase in the percentage of female residents in surgical specialties (p < 0.001) and no significant change in the percentage of female residents in non-surgical specialties. In the same time period, the specialty with the highest percentage of females was Obstetrics & Gynecology (81.3%), and the specialty with the lowest percentage of females was Orthopedic Surgery (13.8%). CONCLUSIONS:Although there has been a positive overall trend in the percentage of females entering medical and surgical specialties, the percentage of females in medicine overall still lies below that of the entire population. Increased efforts are needed to increase female representation in medicine, especially in the U.S. in specialties where they are traditionally underrepresented.

PURPOSE/OBJECTIVE:To report the incidence of immediate (ISBCS) and delayed sequential bilateral cataract surgery (DSBCS) and identify factors associated with undergoing ISBCS. DESIGN/METHODS:Retrospective cohort study. SUBJECTS/METHODS:Medicare beneficiaries aged ≥65 who underwent ISBCS and DSBCS from 2011 to 2019. METHODS:Population-based analysis of the 100% Medicare fee-for-service carrier claims data. Logistic regression models were performed to evaluate factors associated with ISBCS. MAIN OUTCOME MEASURES/METHODS:1) Incidence of ISBCS and DSBCS, 2) demographic, ocular and medical characteristics associated with receipt of ISBCS, and 3) rates of endophthalmitis and cystoid macular edema (CME) after ISBCS or DSBCS. RESULTS:A total of 4,014 (0.2%) ISBCS and 1,944,979 (99.8%) DSBCS patients were identified. Black (OR:2.31, 95%CI: 2.06-2.59), Asian (OR:1.82, 95%CI: 1.51-2.12), or Native American (OR:2.42, 95%CI: 1.81-3.23) patients were more likely to receive ISBCS compared to White patients. Patients residing in rural areas had higher likelihood of ISBCS (OR:1.26, 95%CI: 1.17-1.35) compared to metropolitan areas. Patients operated at a hospital compared to ambulatory setting (OR:2.71, 95%CI: 2.53-2.89) were more likely to receive ISBCS. Patients with bilateral complex vs. non-complex cataract (OR:3.23, 95%CI: 2.95-3.53) were more likely to receive ISBCS. Patients with a Charlson comorbidity index (CCI) of 1-2 (OR:1.45, 95%CI: 1.29-1.62), 3-4 (OR:1.70, 95%CI: 1.47-1.97), 5-6 (OR:1.97, 95%CI: 1.62-2.39), and CCI≥7 (OR:1.97, 95%CI: 1.55-2.50) were all more likely to receive ISBCS compared to those with CCI=0. In contrast, patients with glaucoma (OR:0.82, 95%CI: 0.76-0.89), macular degeneration (OR:0.75, 95%CI: 0.68-0.82), and macular hole/epiretinal membrane (OR:0.55, 95%CI: 0.48-0.65) were less likely to undergo ISBCS compared to those without. Cumulatively, there was no significant difference in endophthalmitis rate within 42 days between ISBCS (1.74 per 1,000 ISBCS) and DSBCS (1.01 per 1,000 DSBCS; p=0.15). Similarly, there was no significant cumulative difference between ISBCS (1.79 per 100 ISBCS) and DSBCS (1.96 per 100 DSBCS) CME rates (p=0.48). CONCLUSION/CONCLUSIONS:Overall utilization of ISBCS among Medicare beneficiaries remains low over the past decade, though rates of endophthalmitis and CME were comparable to DSBCS. Race, geography, systemic and ocular comorbidities were associated with receiving ISBCS. ISBCS represents a potential opportunity to improve access to cataract surgery.

PURPOSE/OBJECTIVE:Since the exact development of posterior subcapsular cataracts (PSCs) is poorly understood, we review various risk factors and propose a two-stage etiology for PSCs. METHODS:The biological mechanisms associated with age-related cataracts (primarily nuclear cataracts, cortical cataracts and PSCs) were reviewed in relation to selected risk factors that induce PSCs (including atopy, diabetes, hypoparathyroidism, myopia, retinitis, solar radiation, steroid use, uveitis, vitrectomy and ionizing radiation). We particularly focused on ionizing radiation, as this is known to be a risk factor specific to PSCs. Based on an analysis of the reviewed material, we propose a detailed explanation of the etiology of PSCs. CONCLUSIONS:Lens epithelial cells (LECs) and lens fiber cells are normally hypoxic and therefore very sensitive to changes in oxidative stress, as quantified by the radiation oxygen effect. We hypothesize that the development of PSC opacities is a two-stage process. Stage I, early in life, is driven by risk factors that promote oxidative stress and ion-pump disruption, harming lens fibers and causing aberrant LECs to proliferate and ectopically migrate as Wedl cells (perhaps by processes associated with an epithelial to mesenchymal transition) to the posterior pole region. After a latent period, in Stage II, the development of PSCs advances mainly due to chronic inflammation and other premature aging-related mechanisms that promote mature vacuolar or plaque PSC. This two-stage hypothesis of PSC etiology accounts for risk factors, such as aging, diabetes and ionizing radiation, which directly affects LECs and the lens. In addition, these risk factors can damage other ocular regions, such as the retina and vitreous, that also indirectly contribute to the development of PSCs. It is possible that the incidence of PSCs may be reduced by reversing the effects of Stage I through various means, including ocular antioxidants.

PURPOSE/OBJECTIVE:To evaluate visual and refractive outcomes and rotational stability of toric intraocular lens (IOL) implantation in eyes with previous ocular surgeries. METHODS:This controlled, longitudinal cohort study included a total of 133 eyes (59 study cases with a history of corneal, vitreoretinal, and/or glaucoma surgery and 74 randomly selected controls without a history of ocular surgery) that had cataract and corneal astigmatism treated with toric IOL implantation. Postoperative outcomes were recorded at postoperative 1 month and 3 to 12 months. RESULTS:Refractive prediction errors were within ±1.00 diopter (D) of target in 93.5% and 88.4% of the study cases at postoperative 1 month and 3 to 12 months, respectively. They were within ±0.50 D of target in 56.5% and 60.5% of the cases during the same follow-up intervals, respectively. Study cases showed statistically significantly inferior uncorrected distance visual acuity (UDVA) compared to controls at 1 month postoperatively (0.27 ± 0.24 and 0.17 ± 0.21 logMAR, respectively, P = .027) but not during the later follow-up (0.19 ± 0.19 and 0.16 ± 0.19 logMAR, respectively, P = .431). Corrected distance visual acuity (CDVA) was slightly lower in the study cases than in controls at 1 month postoperatively (0.13 ± 0.16 and 0.07 ± 0.14, respectively, P = .005) and subsequent follow-up months (0.10 ± 0.13 and 0.03 ± 0.10, respectively, P < .001). Of the examined study cases, 93.9% and 88.4% had IOL axes within 5° of intended axis at postoperative 1 month and 3 to 12 months, respectively. CONCLUSIONS:Toric IOLs provided significant and sustained improvement in visual acuity and refraction in eyes with a history of prior ophthalmic surgery. Refractive outcomes achieved postoperatively were comparable to those in eyes without a prior history of ophthalmic surgery, although the rate of visual recovery may be different. [J Refract Surg. 2019;35(12):781-788.].

OBJECTIVE:To assess the regional variability of corneal endothelial cell density (ECD) between guttae and non-guttae areas in subjects with Fuchs endothelial corneal dystrophy (FECD) using non-contact specular microscopy and confocal microscopy. DESIGN:Retrospective chart review from 2009 to 2014 at the Massachusetts Eye and Ear Infirmary. PARTICIPANTS:One hundred fifteen eyes of 73 subjects with FECD. METHODS:Subjects with FECD underwent same-day specular and confocal microscopy in the same eye. Clinical stage of disease was documented on the day of image acquisition. Regional variability of ECD associated with guttae and non-guttae areas was assessed. Manual endothelial cell counts were performed. RESULTS:; p < 0.0001) as determined by confocal microscopy. CONCLUSION:These findings support confocal microscopy as an alternative to specular microscopy for evaluating the corneal endothelium of patients with FECD, especially those with advanced disease. Confocal microscopy also revealed regional differences in ECD in guttae and non-guttae areas in patients with FECD.