Management of traumatic globe subluxation with optic nerve and extraocular muscle transection.

A care algorithm for partial globe subluxation cases with optic nerve and at least one extraocular muscle (EOM) transection is presented after a literature review was performed using key term variations of globe, ocular, subluxation, optic nerve evulsion or transection, and trauma. Partial globe subluxation cases with transection of the optic nerve and at least 1 EOM were included. Exclusion criteria included globe rupture, complete enucleation defined by a globe without at least 1 EOM attachment, or unclear details confirming optic nerve transection. Including the patient presented herein, a total of 24 patients with 26 eyes were analyzed. About 73.08% of cases underwent initial repositioning (n = 19), with 11.54% of those requiring secondary enucleation or evisceration (n = 3). Of the secondarily managed cases, 2 of the 3 cases listed pain (n = 2) and inadequate cosmesis (n = 1) as rationale. We found that 26.92% of cases underwent initial enucleation (n = 7), citing lack of visual potential and limiting later complications. Most cases favored repositioning, which was typically sustainable. Initial repositioning can improve cosmetic outcome and psychological impact. Given the low risk of later management, cases of traumatic partial subluxation with EOM and optic nerve transections should attempt initial repositioning.

Atypical presentation of invasive myoepithelioma in a pediatric patient.

A 15-year-old girl presented with a mobile lesion with yellowish hue on the posterior lamella of the right lower eyelid adjacent to the punctum. Four years prior, a lesion thought to be a chalazion was excised from the same location. There was subsequent progressive painless enlargement. The patient ultimately underwent an internal excision of the mass. Histopathology demonstrated infiltrative nests and cords of epithelioid and plasmacytoid cells with abundant eosinophilic myxoid-collagenous stroma along with focal infiltration of skeletal muscle with immunohistochemical staining consistent with an invasive myoepithelioma of mixed-cell type. Given concern for invasive disease, the patient underwent subsequent Mohs resection resulting in a 25% full thickness eyelid defect, which was repaired with direct closure of the wedge defect. There has been no recurrence of the disease for 7 months since the Mohs resection. This case illustrates the atypical presentation of an invasive myoepithelioma of the eyelid in a pediatric patient.

Evaluation of a Portable Wavefront Aberrometer for Community Screening Refraction in the Elderly.

SIGNIFICANCE: The SVOne may prove useful to quickly and easily assess refractive correction needs in community screenings and low-resource settings, but not all subjects were testable with the device. PURPOSE: This study aimed to compare the SVOne handheld, smartphone-based wavefront aberrometer with a tabletop autorefractor in identifying refractive errors in elderly subjects. METHODS: Participants 50 years or older at community eye screenings with visual acuity worse than 20/40 in either eye underwent autorefraction followed by two SVOne trials. Power vectors of right eye data were analyzed. RESULTS: Of 84 subjects who underwent autorefraction, 67 (79.8%) were successfully autorefracted with the SVOne, of whom 82.1% (55/67) had a successful repeat reading. Mean M (spherical equivalent) values from tabletop and handheld autorefraction were -0.21 D (95% confidence interval [CI], -0.71 to +0.29 D) and -0.29 D (95% CI, -0.79 to +0.21 D), respectively (P > .05). Mean astigmatism values from tabletop and handheld devices were +1.06 D (95% CI, 0.87 to 1.26 D) and +1.21 D (95% CI, 0.99 to 1.43 D), respectively (P > .05). Intraclass correlation coefficients between devices were 0.95 (95% CI, 0.93 to 0.97) for M, 0.78 (95% CI, 0.66 to 0.86) for J0, and 0.45 (95% CI, 0.24 to 0.63) for J45 (P < .05 for all). Excellent test-retest correlation between SVOne measurements was noted for M (Pearson correlation [r] = 0.96; P < .05), but a weaker correlation was noted for J0 and J45 (r = 0.67 and r = 0.63 [P < .05 for both], respectively). CONCLUSIONS: The SVOne provided strong agreement for M, with the majority of readings within ±1.00 D of each other, when compared with the tabletop autorefractor. A weaker but still good correlation was noted for astigmatism. Similar findings were noted when assessing repeatability.

Basaloid follicular hamartoma of the eyelid in a pediatric patient.

PURPOSE: We report a case a pediatric patient with an eyelid lesion found to be a basaloid follicular hamartoma. OBSERVATIONS: A six-year-old female with juvenile diabetes who presented with a benign eyelid lesion harboring an aberrant eyelash. CONCLUSIONS AND IMPORTANCE: Basaloid follicular hamartoma is a rare benign neoplasm arising from hair follicles. These lesions can resemble basal cell carcinomas and require complete excision.

Pharmacologic pupillary constriction after dilated fundus examination for ectopia lentis to prevent further subluxation.

PURPOSE: To describe two cases of ectopia lentis with different preoperative management strategies following the initial diagnostic dilated exam. OBSERVATIONS: In both cases, the patients presented with bilateral subluxation of the crystalline lens. Neither patient had a known history of Marfan's disease, homocystinuria or other systemic disorders that affect the body's connective tissue possibly leading to lens subluxation. Patient 1 was sent home after dilated fundus examination with no special precautions. That same night, he developed severe right eye pain and further decreased vision. He was found to have complete dislocation of his right crystalline lens into the anterior chamber with corneal edema and an elevated pressure, requiring an urgent pars plana lensectomy. In our second case of ectopia lentis, patient 2 was examined prior to dilation and noted to have bilateral subluxation of the lens into the vitreous. Twenty minutes after dilation, the crystalline lens was noted to be in the anterior chamber in the right eye. The patient was laid supine for several minutes and once the lens was in the posterior cavity, she was given 1% pilocarpine in both eyes to constrict the pupil to prevent dislocation in the anterior chamber. Patient 2 had an uneventful perioperative period and did not suffer worsening subluxation after her initial visit. CONCLUSION AND IMPORTANCE: Unlike patient 1, patient 2 did not suffer further subluxation after her initial dilated eye exam, therefore avoiding a more arduous clinical and surgical course. Patients presenting with completely dislocated lenses may benefit from the reversal of pupillary dilation by being placed in the supine position and given reversal drops such as 1% pilocarpine prior to leaving the office. This method may help prevent complications from anterior lens subluxation and pupillary block glaucoma until definitive surgical managment.

Comparison of self-refraction using a simple device, USee, with manifest refraction in adults.

BACKGROUND: The USee device is a new self-refraction tool that allows users to determine their own refractive error. We evaluated the ease of use of USee in adults, and compared the refractive error correction achieved with USee to clinical manifest refraction. METHODS: Sixty adults with uncorrected visual acuity <20/30 and spherical equivalent between -6.00 and +6.00 diopters completed manifest refraction and self-refraction. RESULTS: Subjects had a mean (±SD) age of 53.1 (±18.6) years, and 27 (45.0%) were male. Mean (±SD) spherical equivalent measured by manifest refraction and self-refraction were -0.90 D (±2.53) and -1.22 diopters (±2.42), respectively (p = 0.001). The proportion of subjects correctable to ≥20/30 in the better eye was higher for manifest refraction (96.7%) than self-refraction (83.3%, p = 0.005). Failure to achieve visual acuity ≥20/30 with self-refraction in right eyes was associated with increasing age (per year, OR: 1.05; 95% CI: 1.00-1.10) and higher cylindrical power (per diopter, OR: 7.26; 95% CI: 1.88-28.1). Subjectively, 95% of participants thought USee was easy to use, 85% thought self-refraction correction was better than being uncorrected, 57% thought vision with self-refraction correction was similar to their current corrective lenses, and 53% rated their vision as "very good" or "excellent" with self-refraction. CONCLUSION: Self-refraction provides acceptable refractive error correction in the majority of adults. Programs targeting resource-poor settings could potentially use USee to provide easy on-site refractive error correction.

Incidence of late atrial fibrillation in bilateral lung versus heart transplants.

BACKGROUND: We compared the incidence of late-onset atrial fibrillation in orthotopic heart transplant recipients and bilateral orthotopic lung transplant recipients. METHODS: We reviewed the records of all heart and lung transplant operations carried out in our institution between 1995 and 2015. We performed 1:1 propensity-matching based on patient age, sex, body mass index, and hypertension. Our primary outcome, late-onset atrial fibrillation, was defined as atrial fibrillation occurring after discharge following hospitalization for transplantation. RESULTS: Over the study period, 397 orthotopic heart transplants and 240 bilateral orthotopic lung transplants were performed. Propensity matching resulted in 173 pairs who were matched with respect to age, sex, body mass index, and preoperative hypertension. The median follow-up was 5.3 years for heart transplant patients and 3.1 years for lung transplant patients. Late-onset atrial fibrillation occurred in 11 heart transplant patients (5 of whom had biopsy-proven evidence of rejection) and 19 lung transplant patients (2 of whom had biopsy-proven evidence of rejection). On Kaplan-Meier analysis, the probability of late-onset atrial fibrillation at 5 years was 4.3% for heart transplant patients vs. 13.9% for lung transplant patients (log-rank p = 0.01). CONCLUSIONS: We documented an increased probability of late-onset atrial fibrillation among bilateral orthotopic lung transplant patients compared to orthotopic heart transplant patients. This was a hypothesis-generating study that suggests a potential role for cardiac autonomic innervation in the genesis of atrial fibrillation.

Duration of Left Ventricular Assist Device Support Does Not Impact Survival After US Heart Transplantation.

BACKGROUND: The aim of this study was to determine whether the duration of left ventricular device support (LVAD) influenced outcomes after orthotopic heart transplantation in a modern, bridge to transplant national cohort. METHODS: The United Network for Organ Sharing database, which has recently made pretransplant LVAD duration available, was queried for all adult bridge to transplant patients between January 2011 and December 2012. Three LVAD duration cohorts were generated, as follows: short (less than 90 days), intermediate (90 to 365 days), and prolonged (more than 365 days). Recipient, donor, and transplant-specific characteristics were compared among the duration cohorts. Unadjusted short-term and long-term survivals were estimated with the Kaplan-Meier method. Risk-adjusted models were also constructed to determine the independent impact of device duration on mortality. RESULTS: Of the 1,332 patients who met criteria for inclusion, 9.8% (n = 130), 54.7% (n = 729), and 35.5% (n = 473) were classified as short, intermediate, and prolonged, respectively. Although the performance status across each cohort was similar at listing (p = 0.38), more patients in the intermediate and prolonged cohorts were considered functionally independent before orthotopic heart transplantation (32% and 37%, respectively, versus 18%; p < 0.001). Additionally, despite worse baseline renal function in the intermediate and prolonged cohorts relative to the short cohort (glomerular filtration rate, 57 and 57 versus 69, p < 0.001), there was no difference in the incidence of new onset posttransplant renal failure (7% versus 10%, 9%, p = 0.41). There was also no difference in 30-day survival (98%, 96%, 95%, p = 0.51), 6-month survival (93%, 92%, 92%, p = 0.93), or 1-year survival (91%, 89%, 89%, p = 0.78) across the cohorts. After risk adjustment, duration did not independently predict mortality at any timepoint. CONCLUSIONS: In the largest, non-industry sponsored study of a modern bridge to transplant cohort, we demonstrated that duration of LVAD support before orthotopic heart transplantation does not influence posttransplant morbidity or mortality. In subanalysis, support for 90 days or more is associated with improvements in pretransplant functional performance.

Characterization of coding synonymous and non-synonymous variants in ADAMTS13 using ex vivo and in silico approaches.

Synonymous variations, which are defined as codon substitutions that do not change the encoded amino acid, were previously thought to have no effect on the properties of the synthesized protein(s). However, mounting evidence shows that these "silent" variations can have a significant impact on protein expression and function and should no longer be considered "silent". Here, the effects of six synonymous and six non-synonymous variations, previously found in the gene of ADAMTS13, the von Willebrand Factor (VWF) cleaving hemostatic protease, have been investigated using a variety of approaches. The ADAMTS13 mRNA and protein expression levels, as well as the conformation and activity of the variants have been compared to that of wild-type ADAMTS13. Interestingly, not only the non-synonymous variants but also the synonymous variants have been found to change the protein expression levels, conformation and function. Bioinformatic analysis of ADAMTS13 mRNA structure, amino acid conservation and codon usage allowed us to establish correlations between mRNA stability, RSCU, and intracellular protein expression. This study demonstrates that variants and more specifically, synonymous variants can have a substantial and definite effect on ADAMTS13 function and that bioinformatic analysis may allow development of predictive tools to identify variants that will have significant effects on the encoded protein.