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Many patients with concussion experience visual symptoms following injury that lead to a diagnosis of convergence insufficiency, accommodative insufficiency, or saccadic dysfunction. However, these diagnostic categories are based on aggregates of clinical tests developed from a non-concussed population and therefore may not accurately describe visual deficits in the concussed population. Thus, we sought to understand individual metrics of visual dysfunction in chronically symptomatic post-concussion patients. This retrospective cross-sectional study included patients examined at the multidisciplinary concussion clinic (MDCC) at Boston Children’s Hospital over four years. Patients aged 5–21 years who had a complete assessment of eye alignment, vergence, accommodation, and visual tracking, and had visual acuity better than or equal to 20/30 in each eye were included. Patients with history of amblyopia, strabismus, or ocular pathology were excluded. Chart review yielded 116 patients who met inclusion criteria (median age 15 years, 64% female). The majority of patients (52%) experienced a single concussion and most were sports-related (50%). Clinical data show vergence, accommodation, or visual tracking deficits in 95% of patients. A receded near point of convergence (NPC, 70/116) and reduced accommodative amplitude (63/116) were the most common deficits. Both NPC and accommodative amplitude were significantly correlated with one another (r = −0.5) and with measures of visual tracking (r = −0.34). Patients with chronic post-concussion symptoms show deficits in individual metrics of vergence, accommodation and visual tracking. The high incidence of these deficits, specifically NPC and accommodative amplitude, highlights the need for a detailed sensorimotor evaluation to guide personalized treatment following concussion.

To create and validate a citywide pediatric Asthma Registry to improve the care and outcomes of children and adolescents in Washington, DC through data-driven quality improvement (QI).All available electronic health record data from inpatient and outpatient domains of Children's National Hospital were aggregated from an existing enterprise data warehouse. Inclusion criteria included asthma relevant ICD-10 codes over the prior 24 months. Available Asthma Registry measures include patient demographics, ambulatory visits, hospital admissions, persistent asthma diagnoses, and prescription of controller medications. Data capture was validated using US Census data and current asthma prevalence estimate of the Behavioral Risk Factor Surveillance System (BRFSS).The registry identified 15,991 DC children and adolescents with asthma aged 0-17 years, inclusive, at the end of 2020. This was 14.2% higher than the estimate of 14,001 children derived from BRFSS. Characteristics of those in the registry included: mean age of 9.5 (1.4) years, 57.9% male, 72.3% Black, and 66.7% publicly insured. Over the prior 24 months, 30.3% had ≥1 emergency department visit, and 10.5% had ≥1 hospital admission. Controller medications were prescribed for 59.6% of children with persistent asthma. Rates varied by sampled primary care practice sites.A population-level pediatric asthma registry captures more children and adolescents with asthma in DC then a BRFSS-derived estimate, and provides city-wide measures of asthma-related utilization. The registry allows for stratification by primary care practice locations and asthma characteristics, supporting the design, implementation, and evaluation of QI projects at the practice, health system, and population levels.Supplemental data for this article can be accessed at publisher's website.

Summary Community transmission of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2 or COVID-19) in the United States on February 26, 2020, and the rapid spread that followed forced patients, providers, payors, and policy makers to adapt to an unprecedented, nearly instant, and enormous demand for virtual care. Although few US ophthalmology practices incorporated telemedicine prior to COVID-19, its use has now become the norm. Regarding the use of synchronous patient-to-provider virtual visits (SPPVV) in pediatric ophthalmology, we have pooled our collective experience at three academic practices across the country to describe initial workflows, technology solutions, use cases, and barriers to care.

Shwachman-Diamond syndrome (SDS) is an autosomal recessive multisystem disorder characterized by exocrine pancreatic dysfunction, bone marrow failure, and leukemia predisposition. Approximately 90% of cases are due to biallelic mutations in the Shwachman-Bodian-Diamond (SBDS) gene. Additional phenotypic features variably associated with SDS include skeletal, neurologic, hepatic, cardiac, endocrine, and dental abnormalities. We report five subjects with SDS who developed a range of inflammatory manifestations. Three patients developed inflammatory eye conditions. Single cases of juvenile idiopathic arthritis, chronic recurrent multifocal osteomyelitis, and scleroderma were also noted. Clinical presentation and treatment responses are described. Proteomic analysis revealed increased inflammatory signatures in SDS subjects as compared to controls. Treatment of inflammatory manifestations in patients with SDS may be complicated by potential myelosuppressive toxicities of anti-rheumatic medications. Further research is needed to better understand the potential link between inflammatory disorders and SDS to inform effective treatment strategies.

PURPOSE To examine the association between Post-Concussion Symptom Scale (PCSS) scores, Convergence Insufficiency Symptom Survey (CISS) scores, and oculomotor deficits post-concussion. METHODS Records of adolescent patients examined in a multidisciplinary concussion clinic between July 2014 and May 2019 were reviewed. PCSS and CISS scores, results of eye examination and oculomotor assessment, concussion history, and demographics were abstracted. RESULTS One hundred and forty patient records (median age, 15.3 years; 52 males, presented 109 days (median) from their most recent concussion) met inclusion criteria. Mean total scores on PCSS and CISS were 46.67 ± 25.89 and 27.13 ± 13.22, respectively, and were moderately correlated with each other (r = 0.53, p

Purpose To determine the frequency of occurrence of receded near point of convergence (NPC) in patients with chronic concussion-related symptoms and in those with receded NPC to enumerate the frequency of convergence insufficiency and other oculomotor disorders. Design Retrospective cross-sectional study. Methods Clinic charts were retrospectively reviewed for the prior 3.5 years to identify all patients 28 days postconcussion, had chronic concussion-related symptoms, had normal visual acuity, and had received a comprehensive sensorimotor examination. The frequency of receded NPC and oculomotor diagnoses were determined. Results Of the 83 eligible patients, 74 (89%) had receded NPC. Of these, 70 (95%) had oculomotor disorders; 30 (41%) had disorders of accommodation only; 21 (28%) had convergence insufficiency and accommodation deficits; and 6 (8%) had convergence insufficiency only. Six (8%) had a convergence deficit other than convergence insufficiency (all with concurrent accommodative disorders); 4 (5%) had both a nonspecific vergence dysfunction and accommodation deficits; 2 (3%) had convergence excess only; and 1 (1%) had both convergence excess and accommodative deficits. Conclusion A receded NPC was present in the majority of young patients with chronic postconcussion symptoms. Associated with numerous underlying oculomotor dysfunctions, the clinical finding of a receded NPC is not synonymous with the diagnosis of convergence insufficiency. Because treatment options for the various oculomotor dysfunctions differ, it is prudent that these patients undergo a thorough examination of their vergence and accommodative systems so that an accurate diagnosis can be made and appropriate treatment prescribed.

Purpose To compare the rates of infectious endophthalmitis following intravitreal injection of ranibizumab using prefilled syringes vs conventional preparation. Design Multicenter retrospective cohort study. Methods All eyes receiving intravitreal injection of 0.5 mg ranibizumab for retinal vascular diseases at 10 retina practices across the United States (2016 to 2017) and Japan (2009 to 2017) were included. The total numbers of eyes and injections were determined from billing codes. Endophthalmitis cases were determined from billing records and evaluated with chart review. Primary outcome was the rate of postinjection acute endophthalmitis. Secondary outcomes were visual acuity and microbial spectrum. Results A total of 243 754 intravitreal 0.5 mg ranibizumab injections (165 347 conventional and 78 407 prefilled) were administered to 43 132 unique patients during the study period. In the conventional ranibizumab group, a total of 43 cases of suspected endophthalmitis occurred (0.026%; 1 in 3845 injections) and 22 cases of culture-positive endophthalmitis occurred (0.013%; 1 in 7516 injections). In the prefilled ranibizumab group, 12 cases of suspected endophthalmitis occurred (0.015%; 1 in 6534 injections) and 2 cases of culture-positive endophthalmitis occurred (0.0026%; 1 in 39 204 injections). Prefilled syringes were associated with a trend toward decreased risk of suspected endophthalmitis (odds ratio 0.59; 95% confidence interval 0.31-1.12; P = .10) and a statistically significant decreased risk of culture-positive endophthalmitis (odds ratio 0.19; 95% confidence interval 0.045-0.82; P = .025). Average logMAR vision loss at final follow-up was significantly worse for eyes that developed endophthalmitis from the conventional ranibizumab preparation compared to the prefilled syringe group (4.45 lines lost from baseline acuity vs 0.38 lines lost; P = .0062). Oral-associated flora was found in 27.3% (6/22) of conventional ranibizumab culture-positive endophthalmitis cases (3 cases of Streptococcus viridans, 3 cases of Enterococcus faecalis) compared to 0 cases in the prefilled ranibizumab group. Conclusion In a large, multicenter, retrospective study the use of prefilled syringes during intravitreal injection of ranibizumab was associated with a reduced rate of culture-positive endophthalmitis, including from oral flora, as well as with improved visual acuity outcomes.

Evidence for hematopoietic stem cell transplantation (HCT) in autoimmune disease has been building since the 1990s; however, many clinicians may not yet be aware of its applications to autoimmune disease. We review the basic tenets of HCT and evidence for autologous HCT in multiple sclerosis (MS), systemic sclerosis (SSc), and lupus with an emphasis on recent advanced phase trials. In MS, the phase 3 randomized MIST trial and the phase 2 randomized ASTIMS trial demonstrated the efficacy of autologous HCT in refractory MS over disease-modifying therapies and mitoxantrone, respectively. In SSc, the phase 3 randomized ASTIS trial and the phase 2 randomized SCOT trial demonstrated the efficacy of autologous HCT in advanced SSc compared to cyclophosphamide. The evidence for HCT in autoimmune diseases continues to grow, particularly in MS and SSc. In lupus, large, comparative trials are still needed. Across autoimmune diseases, questions that still remain to be answered include optimizing patient selection to limit TRM, the appropriate use of MAC, and the necessity for graft manipulation. Furthermore, collaboration between disease-specific and transplant physicians is imperative to expand the appropriate use of HCT in routine clinical practice.

Systemic sclerosis is a chronic autoimmune disease with a poor prognosis, particularly when a patient has rapidly progressive skin or pulmonary involvement. Autologous hematopoietic stem cell transplant is an emerging treatment for this condition, that has been demonstrated to be more effective than immunosuppressants. Careful selection of patients has reduced the transplant-related mortality and maximized the likelihood of benefit. In this report, we present three cases of successful autologous hematopoietic stem cell transplant in patients who would not have met inclusion criteria for entrance into the completed hematopoietic stem cell transplant. After >18 months of follow-up, three patients had clinically significant benefit in terms of skin tightening and pulmonary function tests. Future studies of hematopoietic stem cell transplant in systemic sclerosis may aim to carefully liberalize inclusion criteria to include patients who may not have otherwise been treated while still maintaining an acceptable safety profile.

The authors declare that there are no disclosures or conflicts of interest regarding the publication of this manuscript. We did not receive any financial support and have no financial interests which could create a potential conflict of interest or the appearance of a conflict of interest with regard to the work.

Virtual visits (VVs) are necessitated due to the public health crisis and social distancing mandates due to COVID-19. However, these have been rare in ophthalmology. Over 3.5 years of conducting >350 ophthalmological VVs, our group has gained numerous insights into best practices. This communication shares these experiences with the medical community to support patient care during this difficult time and beyond. We highlight that mastering the technological platform of choice, optimizing lighting, camera positioning, and "eye contact," being thoughtful and creative with the virtual eye examination, and ensuring good documenting and billing will make a successful and efficient VV. Moreover, we think these ideas will stimulate further VV creativity and expertise to be developed in ophthalmology and across medicine. This approach, holds promise for increasing its adoption after the crisis has passed.

Purpose To evaluate the timing of ocular hypertension (OHT) after pediatric closed-globe injury (CGI) and traumatic hyphema. We hypothesize that OHT will occur at different times based on injury characteristics. Design Retrospective, cohort study. Methods Setting: Single-center, tertiary-care, pediatric hospital. Participants: Subjects included patients ≤18 years of age at the time of injury who suffered CGI and traumatic hyphema between 2002 and 2019. Observation Procedure(s): Intraocular pressure and injury demographics were abstracted for every visit after injury. OHT was defined as >21 mm Hg at presentation or after a reading of ≤21 mm Hg at a prior visit. Main Outcome Measures: The primary outcome measure was the timing of OHT categorized into 4 periods: presentation, acute (days 1-7), subacute (days 8-28), or late (day >28). Secondary outcome measures were identification of risks factors for OHT by multivariable logistic regression. Results OHT occurred in 119 of the 305 (39%) subject eyes. OHT occurred in 35 patients at presentation, 69 times acutely, 35 times subacutely, and 36 times late. Pupil damage predicted acute-period OHT (P = .004). OHT at presentation predicted subacute period OHT (P = .004). Iridodialysis and cataract predicted late-period OHT (P = .007 and P Conclusions OHT after CGI and traumatic hyphema in pediatric patients is common. Injury demographics predict this complication. Integration of these risk factors with current literature allows proposal of a risk-stratification tool to guide efficient surveillance for OHT.

Rasmussen encephalitis (RE) is a disorder characterized by drug-resistant seizures and progressive unihemispheric atrophy, hemiparesis, and varying degrees of cognitive decline. The pathophysiology of RE remains elusive, with hypotheses suggesting underlying autoimmune- and T cell-mediated processes. In this case report, we describe a single patient's clinical course from the first day of presentation until definitive treatment for atypical Rasmussen encephalitis at a tertiary care pediatric center. The patient exhibited several atypical features of Rasmussen encephalitis, including a posterior predominance of initial seizure onset with the development of severe choreoathetosis and ipsilateral cerebellar atrophy. He subsequently developed coexistent autoimmune disorders in the form of psoriasis and uveitis, and underwent multiple forms of immunotherapy with limited benefit. This patient shows an association of RE with other autoimmune conditions supporting an autoimmune mechanism of disease while exhibiting several atypical features of RE. Rarely, occipital lobe seizures have been documented as the presenting semiology of this syndrome. This case highlights the need to be mindful of atypical features that may delay hemispherectomy, which remains the definitive treatment. It also suggests that children may be predisposed to the development of autoimmune disorders in later stages of the disease., Highlights • Occipital seizure localization and semiology should not dissuade the diagnosis of Rasmussen's encephalitis • Movement disorders, can accompany Rasmussen's encephalitis • Ipsilateral cerebellar atrophy has been described in Rasmussen's encephalitis • Children with Rasmussen's encephalitis may be predisposed to autoimmune disorders in the later stages of the disease

OBJECTIVE To describe the collaborative findings across a broad array of subspecialties in children and adolescents with postconcussion syndrome (PCS) in a pediatric multidisciplinary concussion clinic (MDCC) setting. DESIGN Retrospective analysis. SETTING Multidisciplinary concussion clinic at a pediatric tertiary-level hospital. PATIENTS Fifty-seven patients seen in MDCC for evaluation and management of PCS between June 2014 and January 2016. INTERVENTIONS Clinical evaluation by neurology, sports medicine, otolaryngology, optometry, ophthalmology, physical therapy, and psychology. MAIN OUTCOME MEASURES Specialty-specific clinical findings and specific, treatable diagnoses relevant to PCS symptoms. RESULTS A wide variety of treatable, specialty-specific diagnoses were identified as potential contributing factors to patients' postconcussion symptoms. The most common treatable diagnoses included binocular vision dysfunction (76%), anxiety, (57.7%), depression (44.2%), new or change in refractive error (21.7%), myofascial pain syndrome (19.2%), and benign paroxysmal positional vertigo (17.5%). CONCLUSIONS Patients seen in a MDCC setting receive a high number of treatable diagnoses that are potentially related to patients' PCS symptoms. The MDCC approach may (1) increase access to interventions for PCS-related impairments, such as visual rehabilitation, physical therapy, and psychological counseling; (2) provide patients with coordinated medical care across specialties; and (3) hasten recovery from PCS.

Purpose: Chromosome 22q11.2 micro-duplication syndrome (MDS), is a rare autosomal dominant condition, with a highly variable phenotype that ranges from unremarkable and asymptomatic, to fatal due to cardiovascular defects. Hypertelorism, downslanting palpebral fissures, superior displacement of the eyebrows, and ptosis are the most commonly reported ocular manifestations. Here, we report a newborn with bilateral exposure, entropion, and corneal ulceration related to 22q11.2 MDS. Observation: A newborn girl presented with bilateral upper eyelid entropion, bilateral lower eyelid ectropion, and lagophthalmos. She subsequently developed bilateral corneal ulcers. Topical antibacterial drops, bandage contact lenses, medroxyprogesterone 1%, and fluorometholone 0.1%, together with partial tarsorrhaphy and correction of eyelid malposition, were used to treat the ulcers and address the underlying issues of exposure and entropion. Genetic testing revealed chromosome 22q11.2.MDS; further evaluation revealed systemic manifestations of this syndrome. The ocular surface healed well with gradual improvement of corneal opacification as well as bilateral partial tarsorrhaphy. Conclusion and importance: This report is the first that describes a newborn with 22q11.2 MDS presenting with sight-threatening corneal ulceration. Entropion, ectropion, and lagophthalmos were identified and treated, allowing for healing of the corneal surface. Genetic testing revealed a syndrome not known to be associated with eyelid abnormalities and corneal ulceration, but with other important systemic and ocular implications. Bilateral partial tarsorrhaphy should not be excluded as a treatment option for infants who fail more conservative measures for the treatment of exposure. Keywords: Chromosome 22q11.2 duplication syndrome, Corneal ulcer, Congenital entropion, Lagophthalmos, Partial tarsorrhaphy

BACKGROUND AND OBJECTIVE: To investigate predictors of visual outcomes in patients who underwent vitrectomy for full-thickness macular hole (FTMH) with at least 1 year of follow-up. PATIENTS AND METHODS: Retrospective, noncomparative, consecutive case series of 132 eyes of 122 patients who underwent surgical repair of idiopathic FTMH with at least 1 year of follow-up. Predictors of visual acuity (VA) outcomes were analyzed using linear regression. RESULTS: Mean follow-up time was 22.2 months. Twenty-three eyes (17.4%) had age-related macular degeneration (AMD), of which 17 (73.9%) cases were mild and nonexudative. At final follow-up, poor preoperative VA ( P < .001), perioperative complications ( P < .001), AMD ( P < .001), and delay from preoperative evaluation to surgery ( P = .037) were significant predictors of final VA. In multiple regression, these variables remained significant ( P < .001, P = .011, P < .001, and P = .002, respectively). CONCLUSION: Poor preoperative VA, perioperative complications, AMD, and delay to surgery were significant predictors of final VA following FTMH repair. [ Ophthalmic Surg Lasers Imaging Retina . 2018;49:566–570.]

Background Posterior ischemic optic neuropathy results from ischemia of the retrobulbar aspect of the optic nerve. It presents as acute loss of vision without optic disc swelling. This is rare in children, with only seven cases reported to date. Neuroimaging is frequently used to aid in the diagnosis of acute visual complaints in children; however, none of the cases described to date delineate the neuroimaging findings of this entity in children. Methods We retrospectively reviewed the electronic medical record. Results We describe the MRI findings in a 10-month-old boy with posterior ischemic optic neuropathy after intraophthalmic artery injection of chemotherapy for retinoblastoma. Conclusions As targeted therapies for retinoblastoma and other diseases amenable to intravascular treatment delivery are more frequently used, the risk of grave vision-related side effects increases. Posterior ischemic optic neuropathy should be considered in the differential diagnosis of any child presenting with acute loss of vision. Dedicated imaging of the orbits can elucidate specific findings that may aid in the diagnosis of this entity in children.

Aims Diabetic retinopathy (DR) and diabetic macular edema (DME) can be evaluated using telemedicine systems, such as the Intelligent Retinal Imaging Systems (IRIS), in patients with Diabetes Mellitus (DM). In an endocrinology-based population utilizing IRIS we determine prevalence rates of DR and DME, and identify associated epidemiologic correlations. Methods This is a multicenter, retrospective chart review using screening data from IRIS. Centers for Disease Control and Prevention (CDC) data on epidemiologic variables (by county) namely, prevalence of DM, incidence of DM, obesity, and time of physical inactivity, were compared against prevalence rates of DR found at screening. Results A total of 10,223 eyes of 5,242 patients with DM were imaged. DR and DME were noted in 1781 (33.98%) and 226 imaging studies (4.31%) respectively. The coefficient of determination was greatest for incidence of DM (R2 = 0.92), followed by DM prevalence (R2 = 0.79), obesity, (R2 = 0.67), and physical inactivity (R2 = 0.34). The presence of DR during screening varied significantly by county (p Conclusions Screening in counties with a higher incidence of DM led to a higher prevalence of identified DR at time of screening. The current work suggests that telemedicine screening in areas known to have a higher incidence of DM may be worthwhile.

Purpose To report the long-term surgical outcomes for a cohort of children with large-angle infantile esotropia. Design Multicenter, nonrandomized clinical study. Methods Setting: Two tertiary-care pediatric hospitals. Study Population: Children with large-angle (≥55 prism diopters) infantile esotropia. Intervention: Surgical treatment of infantile esotropia. Main Outcome Measure: Success rate at final follow-up (postoperative deviation ≤ 10 prism diopters and no need for retreatment). Results A total of 88 patients with large-angle infantile esotropia were treated during the 13-year study period. Treatment was bilateral medial rectus muscle recessions in 70 patients, botulinum toxin–augmented surgery in 15 patients, and 3-muscle surgery in 3 patients. After a mean follow-up of 40 months, 20 patients (23%) had a successful outcome compared to 68 treatment failures (77%). Of the 68 treatment failures, 59 had residual or recurrent esotropia and 9 had sequential exotropia. On multivariate logistic regression, treatment modality was the only factor significantly associated with a successful outcome. Specifically, patients treated with botulinum toxin–augmented surgery were more likely to have a successful outcome compared to patients treated with bilateral medial rectus muscle recessions. For the 26 patients (30%) who underwent retreatment, the mean number of procedures was 2.1, and 7 (27%) had a deviation of ≤10 prism diopters at final follow-up. Conclusions The overall success rate for treatment of large-angle infantile esotropia was poor in this cohort, with most failures owing to recurrent or residual esotropia. Botulinum toxin–augmented surgery was associated with a higher success rate at final follow-up.

BACKGROUND AND OBJECTIVE: The authors evaluated long-term postoperative visual outcomes and recurrence rates following surgery for symptomatic epiretinal membrane (ERM) ± internal limiting membrane (ILM) peeling. PATIENTS AND METHODS: This was a retrospective, consecutive case series of 78 patients undergoing vitrectomy for symptomatic ERM between 1/2010 and 4/2012 with follow-up through at least postoperative year 5 (POY5). Outcomes included visual acuity (VA) (Snellen VA converted to logMAR), central retinal thickness (CRT; μm), and ERM recurrence. Subgroup analysis evaluated outcomes related to ILM peeling. RESULTS: Subgroup analysis based on ILM peeling did not find VA (20/50 [0.430 logMAR ± 0.061 logMAR; mean ± SD] vs. 20/60 [0.518 logMAR ± 0.128 logMAR] for ILM vs. non-ILM peeling respectively; P = .513) nor macular thickness (355 μm ± 13 μm vs. 360 μm ± 42 μm; P = .410) to be significantly different at POY5. Recurrence requiring surgery with and without ILM peeling was not statistically significantly different at POY5 (1.6% and 11.8%; P = .118). CONCLUSION: Vitrectomy for symptomatic ERM led to improved visual and anatomic outcomes with sustained benefit through 5 years. ILM peeling was was associated with reduced ERM recurrence, but this benefit was not statistically significant at POY5. [ Ophthalmic Surg Lasers Imaging Retina . 2018;49:296–302.]

A 37-year-old male with diabetes mellitus and hypertension presented with severe headache. Over a one-month period, magnetic resonance imaging showed acute strokes in the right paramedian pons, left thalamus/globus pallidus/subinsula, and right corona radiata. Evaluation for primary angiitis of the central nervous system (PACNS) included lumbar puncture revealing 7 white blood cells, normal protein and glucose.

To determine whether botulinum toxin is as effective as strabismus surgery in the treatment of acute-onset comitant esotropia in children.Retrospective, nonrandomized, comparative clinical study.Setting: Tertiary care pediatric hospital.Forty-nine children with acute-onset comitant esotropia.Treatment with either botulinum toxin ("chemodenervation group") or standard incisional strabismus surgery ("surgery group").Success rate at 6 months (total horizontal deviation of 10 prism diopters or less and evidence of binocular single vision).There were 16 patients in the chemodenervation group and 33 patients in the surgery group. The success rate was not significantly different at 6 months (81% vs 61%, P = .20) or at 18 months (67% vs 58%, P = .74). The median angle of deviation and median stereoacuity were not significantly different at 6 or 18 months. The chemodenervation procedure was not inferior to incisional strabismus surgery at 6 months. The duration of general anesthesia (5 vs 71 min, P .001) and time in the post-anesthesia care unit (37 vs 93 min, P.001) were significantly shorter in the chemodenervation group. Botulinum toxin injection payment averaged $874 per procedure compared with $2783 for strabismus surgery.Botulinum toxin is at least as effective as surgery in the treatment of acute-onset comitant esotropia at 6 months while reducing the duration of general anesthesia and healthcare costs.

We report a new clinical sign of vitreous inflammation in patients with posterior uveitis: spectral-domain optical coherence tomography identified stalagmite-like, discrete, diffusely distributed, hyperreflective, preretinal deposits in previously vitrectomized eyes of 2 patients during flares of posterior uveitis. The extent of the deposits correlated with disease activity. The underlying primary diseases encountered were necrotizing retinochoroiditis secondary to toxoplasmosis and primary central nervous system lymphoma.

Purpose:With multiple anti–vascular endothelial growth factor (VEGF) and steroid therapies available for diabetic macular edema (DME), there is a need for early determination of the best treatment for a particular patient to prevent irreversible vision loss from chronic DME. In this study, we classify patients as responders or nonresponders to anti-VEGF monotherapy in the treatment of DME after a single anti-VEGF injection.Methods:The study was designed as a single-center, retrospective, interventional case series. We included patients who received 3 consecutive monthly injections with the same anti-VEGF agent. We excluded patients who were treated for DME in the preceding 3 months with any form of anti-VEGF therapy. Visual acuity and central retinal thickness (CRT) data were followed for 1 year. Receiver operating characteristic (ROC) curve analysis was performed in order to identify the cutoff values for identifying responders.Results:One hundred seven eyes were reviewed, with 40 eyes of 34 patients meeting all inclusion criteria. Based on ROC curve analysis, a reduction in CRT by >15% at 1 month identified eyes that responded to treatment and had a >25% reduction in CRT at 3 months (sensitivity, 0.75; specificity, 0.92).Conclusion:Diabetic macular edema eyes that have early response to anti-VEGF treatment by reduction in CRT will have significant response to treatment by 3 months.

Purpose To determine the proportion of patients with proliferative diabetic retinopathy (PDR) who were counted as loss to follow-up (LTFU) patients and to investigate predictive factors. Design Retrospective cohort study. Methods Information was collected for 4,423 patients with PDR between April 30, 2012, and April 30, 2017. Two definitions of LTFU were used. Complete LTFU referred to the population who never returned to care within the study period. Interval LTFU referred to the population who did not adhere to clinical recommendations and missed scheduled appointments, resulting in intervals longer than 6 months or 1 year between 2 appointments. Age, average gross income, and insurance were assessed as potential predictors of interval LTFU. Results Among 4,423 patients with PDR, 2,407 (54.4%) and 2,320 (52.4%) were complete LTFU at 6 months and 1 year, respectively; 782 (17.7%) and 468 (10.6%) patients were interval LTFU for 6 months and 1 year, respectively. Age and average gross income were not found to be significant predictors of interval LTFU. Compared to self-pay, government and private insurance patients were more likely to be interval LTFU at 6 months (government, P = .035; private, P = .005). Private insurance patients were also more likely to be interval LTFU at 1 year (P = .003). Conclusions The identified complete LTFU rates were notably high and warrant further study. More than 1 of 6 patients were interval LTFU for at least 6 months, and 1 of 10 patients was interval LTFU for more than 1 year. Insurance status was significant in determining interval LTFU status. Consistent with other analyses, these results indicate that compliance with clinical appointments among patients with PDR is a substantial clinical challenge.

Purpose: To report the chorioretinal coloboma, and its association with increased risk of retinal detachment (RD) and choroidal neovascularization (CNV). Methods: This retrospective case series included eyes with chorioretinal coloboma diagnosed between 1995 and 2014 with a focus on RD and CNV as related complications. Cases of CNV were managed with laser photocoagulation or intravitreal injection of bevacizumab. For eyes with CNV, therapeutic success was defined as resolution of the subretinal hemorrhage on fundus examination and resolution of the subretinal and intraretinal fluid on optical coherence tomography (OCT). For eyes with RD, anatomic success following surgical intervention was defined as attachment of the retina at the last follow-up visit. Results: Fifty-one eyes of 31 patients with chorioretinal coloboma were identified for review. Bilateral chorioretinal coloboma was present in 64.5% of subjects. RD developed in 15 eyes (29.4%). Among 15 eyes with RD, 4 eyes (27%) had retinal breaks identified within the coloboma, 5 eyes (33%) had retinal breaks outside the coloboma, 2 eyes (13%) showed retinal breaks both inside and outside the coloboma, and in 4 eyes (27%) the causative retinal break was not localized. The overall rate of anatomic success after RD repair was 85.7%. CNV developed in 7 eyes (13.7%) and was located along the margin of the coloboma in all cases. CNV was bilateral in 2 of the 5 affected individuals (40%). Conclusion: RD and CNV were present in a high percentage of eyes with chorioretinal coloboma in these series. The frequent finding of retinal breaks outside the coloboma bed suggests that vitreoretinal interface abnormalities may play a role in development of RD in these eyes.

As seen from both patients’ and physicians’ points of view, there is wide agreement that systemic sclerosis (SSc) is one of the autoimmune disorders with the highest morbidity and mortality rates. In this review, we will follow the evolution of a new approach to its treatment. Support for using hematopoietic stem cell transplantation (HSCT) for SSc arose from seminal studies of genetic and antigen‐induced experimental models of autoimmune disease that demonstrated that high‐dose immunosuppression followed by either allogeneic (same species) or autologous (self) bone marrow transplantation (BMT) could prevent and even reverse damage from autoimmune diseases. Three decades after these initial preclinical observations, our understanding of the therapeutic potential of immune restoration following autologous HSCT has deepened, and the clinical evidence for its application in scleroderma has broadened. In this review we will examine the outcome of conventional therapy for scleroderma lung disease; detail the techniques, toxicities, and results of HSCT for SSc; and explore the biology of immune restoration following autologous transplantation. We will compare the design and outcomes of randomized trials comparing HSCT with cyclophosphamide (CYC) treatment and formulate criteria for the timely referral of patients with scleroderma lung disease for HSCT.

The goal of this review is to provide the reader with an updated summary of the cutaneous manifestations of systemic sarcoidosis, with a particular emphasis on the predilection of sarcoidosis for scars, tattoos, and other areas of traumatized skin. While the mechanism underlying the propensity for traumatized skin to develop sarcoidosis lesions remains unclear, several theories have been proposed including the idea that cutaneous sarcoidosis represents an exuberant, antigen-driven foreign-body response, as well as the theory that traumatized skin represents an immunocompromised district with altered local immune trafficking and neural signaling. In this review, we present two cases in which the development of cutaneous lesions in scars and tattoos was integral to the diagnosis of systemic sarcoidosis. We then review the various cutaneous manifestations of systemic sarcoidosis, the clinical characteristics and differential diagnosis of scar and tattoo sarcoidosis, the proposed mechanism by which traumatized skin is prone to developing sarcoidosis lesions, and current treatments for cutaneous sarcoidosis.

Retinal cavernous hemangiomas are benign vascular anomalies that are typically unilateral and located outside of the macula. Fluorescein angiography findings include an early slow-filling, non-leaking lesion with late intermixed lobules of hyperfluorescence and hypofluoresence secondary to plasma and erythrocyte sedimentation. We present a novel case of unilateral macular cavernous hemangioma with bilateral peripheral vascular anomalies and nonperfusion. This may represent a phenotypic variation of hemangiomas that, in conjunction with recent histopathologic and genetic findings, may aid in future therapies for a disease that has been traditionally observed due to slow progression. [ Ophthalmic Surg Lasers Imaging Retina . 2015;46:764–767.]

A 4-year-old boy presented with a Zone I open globe injury of the left eye. The child was playing with other children at a campground when he was hit directly in the left eye by a wooden stick. The patient was seen at another hospital and transferred to Massachusetts Eye & Ear (MEE) due to concern for open-globe injury. On presentation, the patient was found to have a large, vertical, corneal laceration extending from 12 to 6:30 o’clock with iris prolapse and concern for possible lens violation. The patient was taken to the operating room for exploration and repair of his corneal laceration. During the post-operative period, vision gradually improved, and the lens capsule was noted to be intact. A small posterior subcapsular cataract was noted in the initial post-operative period. Sutures were removed intraoperatively after 1 month due to faster wound healing in most children compared to adults, and the vision continued to improve. The patient followed up with a pediatric and corneal specialist closer to home and continued standard amblyopia therapy with good results.

A 10-year-old male presented with a Zone I corneal laceration with possible lens violation of the left eye on pre-operative evaluation. Given the limitations of the pre-operative examination and intra-operative view of the lens, the globe corneal laceration was repaired primarily without lens extraction. As the corneal edema improved, the view to the lens improved and it was clear the patient had a traumatic cataract with lens violation. Subsequently, secondary cataract extraction and lens implantation was performed with safe lens positioning into the capsular bag 9 days after open globe repair. The patient’s vision slowly improved over time as the corneal wound continued to heal. The pediatric ocular trauma score (POTS) has been devised to predict visual prognosis after pediatric eye injury, and may be a useful tool when counseling families.

A 13-year-old boy presented with a Zone I-II-III open-globe injury of the left eye secondary to an accidental pocketknife injury while at camp. The patient underwent primary repair of the injury, which required hooking and isolating the inferior rectus muscle and repositioning of prolapsed uvea. The patient subsequently required a pars plana vitrectomy, lensectomy, endolaser and membrane peel. With improvement in vision to 20/40 with a contact lens, the patient developed binocular diplopia from a moderate-angle, sensory exotropia. He underwent a left lateral rectus recession and left medial rectus recession with improvement in the cosmetic appearance of his eyes but not complete resolution of his diplopia.

A 6-year old girl presented with a Zone I open globe injury of the right eye from a pair of scissors. Initial evaluation at an outside hospital was notable only for an irregularly shaped pupil, triggering transfer to MEE for further evaluation, where an open globe injury was confirmed. The patient had a 4-mm corneal laceration inferiorly. Intraoperatively iris was found to be trapped in the corneal laceration, requiring use of filtered air and an iris spatula to release the iris tissue. Immediate post-operative visual acuity was poor due to surgically-induced astigmatism from the corneal sutures. Amblyopia therapy was initiated in the post-operative period to maintain optimal visual potential while the corneal laceration healed. Sutures were removed in the operating room at 5 weeks post-operatively. Final visual acuity was 20/15 in both eyes.

An 11-year-old boy presented with a Zone I open-globe injury of the right eye with a piece of pencil graphite embedded in the wound. The patient underwent repair of the open-globe injury with removal of the pencil graphite piece, scraping of the corneal wound edges, and tight wound apposition with 10-0 nylon sutures to promote optimal healing of the round corneal wound. Four weeks following the injury, the corneal sutures were removed in the operating room due to the patient’s age. The patient had a final visual acuity of 20/20 in the injured eye.

Purpose To determine whether botulinum toxin augments the effect of strabismus surgery in pediatric patients with large-angle infantile esotropia. Design Retrospective, comparative, case series. Methods Setting : Tertiary-care pediatric hospital. Study Population : Patients with large-angle infantile esotropia. Intervention : Treatment with botulinum toxin–augmented bilateral medial rectus muscle recessions (“augmented-surgery group”) or traditional bilateral medial rectus muscle recessions (“surgery-only group”). Main Outcome Measure : The effect of surgery on ocular alignment at 4 months, measured in prism diopters of change per mm of surgery (PD/mm). Results There were 14 patients in the augmented-surgery group and 16 patients in the surgery-only group. The mean effect on alignment was significantly greater in the augmented-surgery group compared to the surgery-only group at 4 months (5.7 ± 1.3 vs 4.0 ± 1.4 PD/mm, P = .002) and at 1 year (5.4 ± 1.2 vs 3.7 ± 1.2 PD/mm, P = .002). There was a partial loss of treatment effect between 4 months and 1 year in both groups, which was similar in magnitude (P = .57). On linear regression, there was a trend toward a positive correlation between botulinum toxin dose and treatment effect, but this was not statistically significant (P = .09). Conclusions Botulinum toxin augments the surgical effect of medial rectus muscle recession. Botulinum toxin–augmented surgery may be an alternative to traditional options for large-angle infantile esotropia. A surgical dosing table is proposed for this technique.

To prospectively examine gait characteristics of participants acutely after concussion with and without receded near point of convergence (NPC), compared with healthy controls.Cross-sectional study.Sports-medicine clinic.Patients examined after concussion (n = 33; mean ± SD = 7.2 ± 3.1 days) and a group of uninjured athletes (n = 31) completed a Postconcussion Symptom Scale, underwent NPC testing, and single/dual-task gait assessments.Near point of convergence was defined as the patient-reported diplopia distance when a fixation target moved toward the nose. Receded NPC was defined as a distance5 cm from the tip of the nose.Spatiotemporal gait characteristics in single-task and dual-task conditions were evaluated with analysis of variance; correlations were calculated between NPC and gait measures.Eighteen of 33 (55%) patients with concussion presented with receded NPC. Those with receded NPC exhibited slower gait speed (single-task = 1.06 ± 0.14 m/s vs 1.19 ± 0.15 m/s; dual-task = 0.80 ± 0.13 m/s vs 0.94 ± 0.13 m/s; P = 0.003) and shorter stride lengths (single-task = 1.11 ± 0.10 m vs 1.24 ± 0.11 m; dual-task = 0.97 ± 0.11 m vs 1.09 ± 0.11 m; P = 0.001) than healthy controls. Near point of convergence was moderately correlated with dual-task average walking speed for the normal NPC group (ρ = -0.56; P = 0.05). Postconcussion Symptom Scale scores did not significantly differ between groups (27 ± 18 vs 28 ± 16).After concussion, adolescents with receded NPC exhibited significant gait-related deficits compared with healthy controls, whereas those with normal NPC did not. Vergence and gross motor system dysfunction may be associated after concussion. Gait and vergence measures may contribute useful information to postconcussion evaluations.

Objective Pyridoxine is converted to its biologically active form pyridoxal-5-phosphate (P5P) by the enzyme pyridox(am)ine 5′-phosphate oxidase and serves as a cofactor in nearly 200 reactions in the central nervous system. Pyridox(am)ine 5′-phosphate oxidase deficiency leads to P5P dependent epilepsy, typically a neonatal- or infantile-onset epileptic encephalopathy treatable with P5P or in some cases, pyridoxine. Following identification of retinopathy in a patient with pyridox(am)ine 5′-phosphate oxidase deficiency that was reversible with P5P therapy, we describe the systemic manifestations of pyridox(am)ine 5′-phosphate oxidase deficiency. Methods A series of six patients with homozygous mutations of PNPO , the gene coding pyridox(am)ine 5′-phosphate oxidase, were evaluated in our center over the course of two years for phenotyping of neurological and systemic manifestations. Results Five of six were born prematurely, three had anemia and failure to thrive, and two had elevated alkaline phosphatase. A movement disorder was observed in two children, and a reversible retinopathy was observed in the most severely affected infant. All patients had neonatal-onset epilepsy and were on a continuum of developmental delay to profound encephalopathy. Electroencephalographic features included background slowing and disorganization, absent sleep features, and multifocal and generalized epileptiform discharges. All the affected probands carried a homozygous PNPO mutation (c.674 G>T, c.686 G>A and c.352G>A). Conclusion In addition to the well-described epileptic encephalopathy, pyridox(am)ine 5′-phosphate oxidase deficiency causes a range of neurological and systemic manifestations. A movement disorder, developmental delay, and encephalopathy, as well as retinopathy, anemia, and failure to thrive add to the broadening clinical spectrum of P5P dependent epilepsy.

Purpose To evaluate the effect of a computer-based training program—Massachusetts Eye & Ear ROP Trainer—on residents' knowledge of retinopathy of prematurity (ROP) management. Methods In this prospective, randomized study, ophthalmology residents from nine different training programs consented to participate. Those who completed the study were randomly assigned to either the Trainer or the control group. The ROP Trainer was created using clinical cases encompassing the stages of ROP in digital pictures and videos. It includes sections on screening decisions, examination techniques, and diagnosis, and a reference section with the expert video clips and a searchable image library. Subjects in the control group were asked to study standard print material on ROP. A pre- and post-test, consisting of theoretical and practical (diagnosis) questions, and a post-intervention satisfaction test were administered. Accuracy of ROP diagnosis was assessed. Results A total of 180 residents agreed to participate, of whom 60 completed the study. Residents in the Trainer group had statistically significant improvements (P = 0.003) in ROP knowledge and diagnostic ability (P = 0.005). Residents randomized to the Trainer group were more satisfied with the training materials than were those in the control group. There was no significant difference in improving knowledge by year of training, sex, or country. Considering all training levels, a statistically significant increase was observed in sensitivity for the diagnosis of preplus or worse, zone I or II, ROP stage, category, and aggressive posterior ROP in the Trainer group. Conclusions In this study, the Trainer was shown to significantly improve ROP knowledge and diagnostic skills of residents, regardless of sex, year, of training, or country.

We present an 8-month-old boy with severe retinal detachment from familial exudative vitreoretinopathy ( FZD4 exon 1 deletion). He was subsequently diagnosed with spinal muscular atrophy with SMN1 deletion. β-catenin signaling is dysregulated in both disorders, so we hypothesize that the co-occurrence may have exacerbated the vitreoretinal phenotype.

Patients undergoing gastric bypass procedures are predisposed to many vitamin and mineral deficiencies including copper, which can lead to optic neuropathy.A 35-year-old woman complained of progressive vision loss bilaterally 3 years after gastric bypass surgery (GBS).Ophthalmic examination revealed the presence of subnormal visual acuity and pale optic nerves bilaterally. Laboratory testing showed copper deficiency. Complete improvement in vision was noted after copper was replenished.Nutritional deficiencies occurring after gastric bypass procedures are well reported. They happen and occur due to reduced gastric and enteral absorption and cause many systemic and neurologic manifestations. Optic neuropathy occurring after GBS is often due to vitamin B12 and folic acid deficiency. Copper deficiency causing systemic symptoms is very rare and usually takes decades to manifest. Our case is unique in that copper deficiency was noted as early as 3 years after GBS. Moreover rapid copper replenishment led to a dramatic recovery of vision. Our case underscores the need for maintaining high suspicion when evaluating patients with suspected optic neuropathy occurring after GBS since prompt diagnosis and treatment may lead to reversal of visual loss.

Evaluate predictors and outcomes of ocular hypertension after open-globe injury.This is a retrospective, case-control study reviewing records of consecutive patients with open-globe injuries treated at Massachusetts Eye and Ear Infirmary between February 1999 and January 2007. Of 658 patients treated, 382 had at least 2 months of follow-up and sufficient data to be included. Main outcome measures are visual acuity, intraocular pressure (IOP), and type of glaucoma intervention employed.Sixty-five (17%) patients developed ocular hypertension defined as IOP≥22 mm Hg at1 visit or requiring treatment. Increased age (P0.001), hyphema (0.025), lens injury (P0.0001), and zone II injury (P=0.0254) are risk factors for developing ocular hypertension after open-globe injury. Forty-eight (74%) patients with ocular hypertension were treated medically, 8 (12%) underwent filtering or glaucoma drainage device surgery, 5 (8%) had IOP normalization with observation, while 4 (6%) required anterior chamber washout with no other glaucoma surgery. Patients with ocular hypertension had an average maximum IOP=33.4 mm Hg at a median follow-up of 21 days, with most patients maintaining normal IOP at all follow-up time points. Visual acuity improved over time with median acuity of hand motions preoperatively, and 20/60 at 12 and 36 months.Ocular hypertension is a significant complication after open-globe injury that sometimes requires surgical intervention. Predictive factors can alert physicians to monitor for elevated IOP in the first month after trauma. Most patients with traumatic ocular hypertension had improved visual acuity and IOP normalization over time.