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  • Choroidal neovascularization in a child with infantile primary hyperoxaluria treated with bevacizumab JOURNAL OF AAPOS Michalak, S. M., Bonafede, L., Kelly, M. P., Cai, C. X., Chen, X. 2021; 25 (2): 128-131


    Fundus manifestations of primary hyperoxaluria include crystalline deposits, focal or diffuse macular hyperpigmentation, and subretinal fibrosis. Choroidal neovascularization has been hypothesized to underlie the pathogenesis of subretinal fibrosis, yet its manifestations are rarely observed. We report a case of infantile primary hyperoxaluria type 1 in a 17-month-old infant with macular subretinal fluid and subretinal hemorrhage that was associated with leakage on fluorescein angiography and responded to bevacizumab treatment, consistent with choroidal neovascularization. This case suggests that choroidal neovascularization may contribute to subretinal fibrosis and subsequent vision loss in infantile primary hyperoxaluria and may benefit from anti-vascular endothelial growth factor therapy.

    View details for DOI 10.1016/j.jaapos.2020.12.004

    View details for Web of Science ID 000665589700028

    View details for PubMedID 33737053

  • Effect of ICD-9 to ICD-10 Transition on Accuracy of Codes for Stage of Diabetic Retinopathy and Related Complications: Results from the CODER Study OPHTHALMOLOGY RETINA Cai, C. X., Michalak, S. M., Stinnett, S. S., Muir, K. W., Fekrat, S., Borkar, D. S. 2021; 5 (4): 374-380


    When the International Classification of Diseases 9th Revision (ICD-9) transitioned to the International Classification of Diseases 10th Revision (ICD-10), there was a marked increase in the complexity of International Classification of Diseases (ICD) codes with potential for improved specificity in clinical database research. The purpose of this study was to characterize the accuracy of coding for stage of diabetic retinopathy (DR) and DR-related complications (including vitreous hemorrhage, retinal detachment, and neovascular glaucoma) during this transition.Retrospective chart review of 3 time periods corresponding to the use of ICD-9: 2014-2015; "early" use of ICD-10, 2015-2016; and "late" use of ICD-10, 2018-2019.Patients aged 18 years or older with a diagnosis of DR at a multispecialty academic institution.Positive predictive value (PPV), negative predictive value (NPV), sensitivity, specificity, and kappa (κ) statistics were generated for each diagnosis. Generalized estimating equation (GEE) models were used to assess the significance of the variables.The main outcome was the proportion of agreement between the ICD code and the documented chart standard for stage of DR and DR-related complications.A total of 600 patients were included in the study (average age, 61 years; range, 25-93 years). Overall, there was substantial agreement between the ICD codes for stage of DR and the documented standard (κ = 0.66). The proportion of ICD codes in agreement with the documented standard diagnosis increased with time: 66.5%, 78.5%, and 83.3% for ICD-9, "early" ICD-10, and "late" ICD-10, respectively. The odds of agreement were 2.67 (95% confidence interval [CI], 1.49-4.76, P < 0.001) and 3.96 (95% CI, 2.34-6.69, P < 0.0001) times greater for "early" and "late" ICD-10 codes compared with ICD-9 codes, respectively. For specific codes, the overall PPV, NPV, sensitivity, and specificity for nonproliferative diabetic retinopathy (NPDR) and proliferative diabetic retinopathy (PDR) were excellent (>90%). The odds of agreement were 19.70 (95% CI, 11.54-33.64, P < 0.0001) times greater for PDR than NPDR. Compared with the stage of DR, DR-related diagnoses were overall less accurately coded (κ = 0.61, 0.48, and 0.52 for vitreous hemorrhage, retinal detachment, and neovascular glaucoma, respectively).Coding in ICD-10 is more accurate than in ICD-9, particularly for PDR compared with NPDR. The increased accuracy emphasizes the potential for ICD-10 coding to be used effectively in database research.

    View details for DOI 10.1016/j.oret.2020.08.004

    View details for Web of Science ID 000664195400014

    View details for PubMedID 32810681

  • Identifying Characteristics Predictive of Lost-to-Follow-Up Status in Amblyopia. American journal of ophthalmology Shoshany, T. N., Chinn, R. N., Staffa, S. J., Bishop, K., Michalak, S., Hunter, D. G. 2021; 230: 200-206


    To identify demographic and disease-related characteristics predictive of Lost-to-Follow-Up (LTFU) status in amblyopia treatment and create a risk model for predicting LTFU status.Retrospective cohort study METHODS: Setting: Single-center, ophthalmology department at Boston Children's Hospital (BCH).2037 patients treated for amblyopia at BCH between 2010 and 2014.LTFU was defined as patients who did not return after initial visit, excluding those who came for second opinion. Multiple variables were tested for association with LTFU status.Odds ratio of LTFU risk associated with each variable. Multivariate logistic regression was used to create a risk score for predicting LTFU status.A large proportion of patients (23%) were LTFU after first visit. Older age, nonwhite race, lack of insurance, previous glasses or atropine treatment, and longer requested follow-up intervals were independent predictors of LTFU status. A multivariable risk score was created to predict probability of LTFU (area under the curve 0.68).Our comprehensive amblyopia database allows us to predict which patients are more likely to be LTFU after baseline visit and develop strategies to mitigate these effects. These findings may help with practice efficiency and improve patient outcomes in the future by transitioning these analyses to an electronic medical record that could be programmed to provide continually updated decision support for individual patients based on large data sets.

    View details for DOI 10.1016/j.ajo.2021.05.002

    View details for PubMedID 33992614

  • Effect of Sequential and Simultaneous Patching Regimens in Unilateral Amblyopia. American journal of ophthalmology Chinn, R. N., Michalak, S. M., Shoshany, T. N., Bishop, K., Staffa, S. J., Hunter, D. G. 2021


    Many clinicians treat unilateral amblyopia with glasses alone and initiate patching when needed; others start glasses and patching simultaneously. In this study, we reviewed the outcomes of the two approaches at our institution.Retrospective non-randomized clinical trial METHODS: Setting: Institutional practice.All patients diagnosed with amblyopia at Boston Children's Hospital between 2010 and 2014.Unilateral amblyopia visual acuity (VA): 20/40-20/200 with interocular difference ≥ 3 lines, age 3-12 years, with a 6-month visit.Deprivation amblyopia, prior amblyopia treatment, treatment other than patching, surgery. Patients were categorized as "simultaneous treatment" (concurrent glasses and patching therapy at their first visit) or "sequential treatment" (glasses alone at first visit followed by patching therapy at second visit.) Observation Procedures: Patient demographics, VA, and stereopsis were compared.VA and stereopsis at the last visit on treatment.We identified 98 patients who met inclusion criteria: 36 received simultaneous treatment and 62 sequential treatment. Median amblyopic eye VA improved similarly between the simultaneous (∆0.40 (0.56, 0.30 logMAR) and sequential (∆0.40 (0.52, 0.27 logMAR) groups. Patients without stereopsis at first visit had better stereopsis outcomes with sequential treatment (5.12 (4.00, 7.51) log stereopsis) compared to simultaneous treatment (8.01 (5.65, 9.21) log stereopsis, p ≤ 0.046).VA improved approximately 4 lines regardless of treatment type. For children without stereopsis at first presentation, sequential patching yielded better stereopsis outcomes. These findings require further validation and highlight the importance of evaluating stereopsis in future studies.

    View details for DOI 10.1016/j.ajo.2021.07.012

    View details for PubMedID 34303687

  • The effect of nonmodifiable physician demographics on Press Ganey patient satisfaction scores in ophthalmology JOURNAL OF AAPOS Michalak, S. M., Bhullar, P. K., Enyedi, L. B. 2020; 24 (5): 299-301


    Press Ganey patient satisfaction scores are increasingly being used as a physician quality metric. In this retrospective review of over 25,000 patient surveys for 61 ophthalmologists, pediatric ophthalmologists and neuro-ophthalmologists received significantly lower patient satisfaction scores than their peers, suggesting that the problem for which a patient seeks care may affect whether he or she is satisfied with the care received. These findings should be taken into account when considering the validity of Press Ganey scores as an equitable, modifiable measure of physician performance, especially when considering factoring these scores into physician evaluation and reimbursement.

    View details for DOI 10.1016/j.jaapos.2020.05.010

    View details for Web of Science ID 000601378800018

    View details for PubMedID 32890735

  • Optic Nerve Edema, Venous Stasis Retinopathy, and Peripheral Retinal Whitening in a Teenage Girl JAMA OPHTHALMOLOGY Michalak, S. M., Meekins, L. C., Ali, M. H. 2020; 138 (10): 1087-1088
  • Evaluating Amblyopia Treatment Success Using the American Academy of Ophthalmology IRIS50 Measures OPHTHALMOLOGY Shoshany, T. N., Michalak, S. M., Chinn, R. N., Staffa, S. J., Hunter, D. G. 2020; 127 (6): 836-838

    View details for Web of Science ID 000553874500020

    View details for PubMedID 32199623

  • Effect of Primary Occlusion Therapy in Asymmetric, Bilateral Amblyopia AMERICAN JOURNAL OF OPHTHALMOLOGY Shoshany, T. N., Michalak, S., Staffa, S. J., Chinn, R. N., Bishop, K., Hunter, D. G. 2020; 211: 87-93


    Many bilateral amblyopia patients have asymmetric visual acuity (VA). There is no standard treatment for these patients, and outcomes have not been well described. Our goal is to compare VA outcomes in this group based on timing of occlusion therapy.Retrospective interventional comparative case series.Setting: Institutional practice. PatientPopulation: Patients diagnosed with amblyopia at Boston Children's Hospital between 2010 and 2014. InclusionCriteria: VA ≥ 0.3 logMAR bilaterally by objective optotype-based measures, interocular difference (IOD) ≥ 0.18 logMAR, age 2-12 years. ExclusionCriteria: Loss to follow-up, managed surgically, deprivation amblyopia. Patients had either primary or secondary occlusion (primary = initiated when VA ≥ 0.3 logMAR bilaterally; secondary = initiated to correct residual IOD once VA improved to ≤0.18 logMAR in the stronger eye). ObservationProcedure: Patient demographics, VA, IOD, and stereopsis were compared between groups. OutcomeMeasures: VA improvement at 12-18 months and at last visits.Of 2,200 patients reviewed, 167 (7.6%) had asymmetric, bilateral amblyopia; 98 met inclusion and exclusion criteria. Patients were equally divided between primary (n = 50) and secondary (n = 48) occlusion groups. There were no differences in demographics, baseline VA, or IOD between groups (P ≥ .22), although the primary occlusion group had a higher proportion of strabismic amblyopia (P = .007). VA in both eyes, IOD, and stereopsis improved similarly between groups, even after stratifying by amblyopia subtype (P ≥ .48). The secondary occlusion group was more likely to achieve 20/30 bilaterally and IOD ≤ 1 line at 12-18 months (P ≤ .4), although this equalized by the last visit.In patients with asymmetric, bilateral amblyopia, VA improved by 4 lines in the weaker eye and 2 lines in the stronger eye, while IOD improved by 2 lines, irrespective of occlusion status. Primary occlusion thus provided no further benefit over spectacle correction alone.

    View details for DOI 10.1016/j.ajo.2019.10.030

    View details for Web of Science ID 000522799700010

    View details for PubMedID 31712066

  • BILATERAL PARACENTRAL ACUTE MIDDLE MACULOPATHY AFTER CARDIOPULMONARY BYPASS. Retinal cases & brief reports Michalak, S. M., Mukherjee, N., Gospe, S. M. 2020


    To report the first two observations of bilateral paracentral acute middle maculopathy in the early postoperative period after cardiopulmonary bypass procedures.Comprehensive ophthalmic examinations were performed, including best-corrected visual acuity, Humphrey visual field testing, dilated fundus examination, and optical coherence tomography.Two patients complained of unilateral vision loss after cardiac surgery involving cardiopulmonary bypass. Unilateral optic disc edema consistent with ischemic optic neuropathy was observed in the symptomatic eye of each patient, whereas scattered cotton-wool spots were identified bilaterally. In addition, optical coherence tomography imaging revealed bilateral paracentral acute middle maculopathy, correlating with visual field defects detected on automated perimetry in the asymptomatic eyes.Although symptomatic postoperative vision loss is a rare complication of cardiac surgery, intraoperative retinal microvascular nonperfusion as a consequence of extracorporeal circulation of blood may be a more common and under-recognized occurrence.

    View details for DOI 10.1097/ICB.0000000000000982

    View details for PubMedID 32032289

  • Is Serial Electroneuronography Indicated Following Temporal Bone Trauma? OTOLOGY & NEUROTOLOGY Remenschneider, A., Michalak, S., Kozin, E. D., Barber, S., De Venecia, R. K., Hadlock, T. A., Jung, D. H. 2017; 38 (4): 572-576


    Contemporary guidelines advise facial nerve (FN) decompression within 2 weeks of temporal bone trauma if a single electroneuronography (ENoG) demonstrates more than 90% degeneration of the FN. We report a case series demonstrating the potential of serial ENoG to guide FN management more than 2 weeks following injury.Adults with traumatic temporal bone fractures and resultant ipsilateral FN paresis.Serial ENoG followed by observation or decompression of the FN.House-Brackmann (HB) graded FN function.Nine cases of blunt temporal bone trauma resulting in ispilateral FN paralysis were identified and reviewed. Two patients were women, and average age at the time of trauma was 30 years (range, 17-52). Immediate paralysis occurred in four cases, while five were delayed. A single ENoG was performed in seven patients and was predictive of final function in six, while one patient had an initially reassuring ENoG but did not obtain full recovery of FN function (HB 4). Two patients underwent serial ENoG on a weekly basis which, while initially reassuring, demonstrated declining FN function on subsequent testing. Decompression was performed in both patients with excellent recovery of FN function (HB1 and HB2).The majority of ENoGs performed within 2 weeks of temporal bone trauma provide sufficient prognostic data for treatment decisions; however, in selected cases, a single ENoG may not adequately predict long-term FN outcomes. For patients failing to improve with observation alone, serial ENoG may capture declining FN function, identifying patients that may benefit from late decompression.

    View details for DOI 10.1097/MAO.0000000000001337

    View details for Web of Science ID 000397773100020

    View details for PubMedID 28114180

  • Ocular Motor Nerve Development in the Presence and Absence of Extraocular Muscle INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE Michalak, S. M., Whitman, M. C., Park, J. G., Tischfield, M. A., Nguyen, E. H., Engle, E. C. 2017; 58 (4): 2388-2396


    To spatially and temporally define ocular motor nerve development in the presence and absence of extraocular muscles (EOMs).Myf5cre mice, which in the homozygous state lack EOMs, were crossed to an IslMN:GFP reporter line to fluorescently label motor neuron cell bodies and axons. Embryonic day (E) 11.5 to E15.5 wild-type and Myf5cre/cre:IslMN:GFP whole mount embryos and dissected orbits were imaged by confocal microscopy to visualize the developing oculomotor, trochlear, and abducens nerves in the presence and absence of EOMs. E11.5 and E18.5 brainstems were serially sectioned and stained for Islet1 to determine the fate of ocular motor neurons.At E11.5, all three ocular motor nerves in mutant embryos approached the orbit with a trajectory similar to that of wild-type. Subsequently, while wild-type nerves send terminal branches that contact target EOMs in a stereotypical pattern, the Myf5cre/cre ocular motor nerves failed to form terminal branches, regressed, and by E18.5 two-thirds of their corresponding motor neurons died. Comparisons between mutant and wild-type embryos revealed novel aspects of trochlear and oculomotor nerve development.We delineated mouse ocular motor nerve spatial and temporal development in unprecedented detail. Moreover, we found that EOMs are not necessary for initial outgrowth and guidance of ocular motor axons from the brainstem to the orbit but are required for their terminal branching and survival. These data suggest that intermediate targets in the mesenchyme provide cues necessary for appropriate targeting of ocular motor axons to the orbit, while EOM cues are responsible for terminal branching and motor neuron survival.

    View details for DOI 10.1167/iovs.16-21268

    View details for Web of Science ID 000400649600051

    View details for PubMedID 28437527

    View details for PubMedCentralID PMC5403115

  • Incidence and Predictors of Complications and Mortality in Cerebrovascular Surgery: National Trends From 2007 to 2012 NEUROSURGERY Michalak, S. M., Rolston, J. D., Lawton, M. T. 2016; 79 (2): 182-192


    Cerebrovascular surgery offers potentially lifesaving treatments for intracranial vascular pathology yet bears substantial risks in the form of perioperative complications and mortality.To better characterize the risks associated with cerebrovascular surgery by broadly investigating the incidence of complications, patient-level predictors of complications, and mortality using the National Surgical Quality Improvement Program database, a prospective, audited, national data set.All cerebrovascular cases were extracted from the National Surgical Quality Improvement Program with the use of Current Procedural Terminology codes. Complication and mortality rates were analyzed with univariate and multivariate statistical analyses.A total of 1141 cases were analyzed. The rate of complications was nearly twice that of previous estimates: Almost one-third of patients (30.9%) experienced at least 1 complication, which was significantly associated with 30-day mortality (odds ratio, 7.76; 95% confidence interval, 4.27-14.10; P < .001). Emergency surgery was associated with higher mortality rates (15.1%) than nonemergency procedures (2.3%). Significant predictors of complications included preoperative ventilator dependence, emergency surgery, bleeding disorders, diabetes mellitus, and alcohol abuse. Significant predictors of mortality included postoperative coma >24 hours, preoperative or postoperative ventilator dependence, black or Asian race, and stroke. The most common complications were ventilator dependence (64.5% in patients ventilated preoperatively, 8.4% in patients not ventilated preoperatively), bleeding requiring transfusion (10.2%), reoperation within 30 days (9.6%), pneumonia (7.3%), and stroke (7.3%).Cerebrovascular surgery is associated with significant risks of morbidity and mortality. Mitigation of these risks requires broader, patient-centered understanding of risk factors and complications specific to cerebrovascular surgery, as presented in this article. These findings pave the way for improving patient safety and outcomes in cerebrovascular surgery.AVM, arteriovenous malformationCI, confidence intervalNSQIP, National Surgical Quality Improvement ProjectOR, odds ratio.

    View details for DOI 10.1227/NEU.0000000000001251

    View details for Web of Science ID 000382335100017

    View details for PubMedID 27171325

  • Prospective, multidisciplinary recording of perioperative errors in cerebrovascular surgery: is error in the eye of the beholder? JOURNAL OF NEUROSURGERY Michalak, S. M., Rolston, J. D., Lawton, M. T. 2016; 124 (6): 1794-1804


    OBJECT Surgery requires careful coordination of multiple team members, each playing a vital role in mitigating errors. Previous studies have focused on eliciting errors from only the attending surgeon, likely missing events observed by other team members. METHODS Surveys were administered to the attending surgeon, resident surgeon, anesthesiologist, and nursing staff immediately following each of 31 cerebrovascular surgeries; participants were instructed to record any deviation from optimal course (DOC). DOCs were categorized and sorted by reporter and perioperative timing, then correlated with delays and outcome measures. RESULTS Errors were recorded in 93.5% of the 31 cases surveyed. The number of errors recorded per case ranged from 0 to 8, with an average of 3.1 ± 2.1 errors (± SD). Overall, technical errors were most common (24.5%), followed by communication (22.4%), management/judgment (16.0%), and equipment (11.7%). The resident surgeon reported the most errors (52.1%), followed by the circulating nurse (31.9%), the attending surgeon (26.6%), and the anesthesiologist (14.9%). The attending and resident surgeons were most likely to report technical errors (52% and 30.6%, respectively), while anesthesiologists and circulating nurses mostly reported anesthesia errors (36%) and communication errors (50%), respectively. The overlap in reported errors was 20.3%. If this study had used only the surveys completed by the attending surgeon, as in prior studies, 72% of equipment errors, 90% of anesthesia and communication errors, and 100% of nursing errors would have been missed. In addition, it would have been concluded that errors occurred in only 45.2% of cases (rather than 93.5%) and that errors resulting in a delay occurred in 3.2% of cases instead of the 74.2% calculated using data from 4 team members. Compiled results from all team members yielded significant correlations between technical DOCs and prolonged hospital stays and reported and actual delays (p = 0.001 and p = 0.028, respectively). CONCLUSIONS This study is the only of its kind to elicit error reporting from multiple members of the operating team, and it demonstrates error is truly in the eye of the beholder-the types and timing of perioperative errors vary based on whom you ask. The authors estimate that previous studies surveying only the attending physician missed up to 75% of perioperative errors. By finding significant correlations between technical DOCs and prolonged hospital stays and reported and actual delays, this study shows that these surveys provide relevant and useful information for improving clinical practice. Overall, the results of this study emphasize that research on medical error must include input from all members of the operating team; it is only by understanding every perspective that surgical staff can begin to efficiently prevent errors, improve patient care and safety, and decrease delays.

    View details for DOI 10.3171/2015.5.JNS142458

    View details for Web of Science ID 000376476200033

    View details for PubMedID 26636377

  • Shape Beyond Recognition: Form-Derived Directionality and Its Effects on Visual Attention and Motion Perception JOURNAL OF EXPERIMENTAL PSYCHOLOGY-GENERAL Sigurdardottir, H. M., Michalak, S. M., Sheinberg, D. L. 2014; 143 (1): 434-454


    The shape of an object restricts its movements and therefore its future location. The rules governing selective sampling of the environment likely incorporate any available data, including shape, that provide information about where important things are going to be in the near future so that the object can be located, tracked, and sampled for information. We asked people to assess in which direction several novel objects pointed or directed them. With independent groups of people, we investigated whether their attention and sense of motion were systematically biased in this direction. Our work shows that nearly any novel object has intrinsic directionality derived from its shape. This shape information is swiftly and automatically incorporated into the allocation of overt and covert visual orienting and the detection of motion, processes that themselves are inherently directional. The observed connection between form and space suggests that shape processing goes beyond recognition alone and may help explain why shape is a relevant dimension throughout the visual brain.

    View details for DOI 10.1037/a0032353

    View details for Web of Science ID 000331298600038

    View details for PubMedID 23565670

    View details for PubMedCentralID PMC3726554