Surgical Management of Overgrowth Syndrome With Bilateral Vision-Threatening Ptosis.

We describe the first case reported in ophthalmological literature of the surgical management of a 17-month-old boy with bilateral vision-threatening ptosis, tarsomegaly, ectropion, and euryblepharon secondary to suspected overgrowth syndrome. We elaborate on the major challenges associated with surgical management including the natural and asymmetric growth of oversized tissue, the high likelihood of scarring and formation of disorganized tissue, and risks of frequent intubation in these patients who may have lesions that compromise critical structures such as the airway. Ultimately, surgical intervention is encouraged primarily if vision or ocular health is threatened and secondarily to achieve good cosmesis.

Epidemiological trends and survival outcomes for dermatofibrosarcoma protuberans of the head and neck region.

BACKGROUND: Given the rarity of dermatofibrosarcoma protuberans (DFSP) of the head and neck, the incidence and prognosis specific to this region are poorly defined. The purpose of this study was to determine epidemiology, clinicopathological characteristics, and prognostic factors of patients with DFSP of the head and neck region, using the Surveillance, Epidemiology, and End Results (SEER) database. METHODS: A cohort analysis was performed for primary head and neck DFSP reported to the SEER database between 2000 and 2018. Overall survival was determined using Kaplan-Meier analysis while Cox regression modeling was used to examine predictive factors. RESULTS: A total of 681 cases were reported to the SEER database between 2000 and 2018. Incidence rates decreased over time. Overall survival was 94%, and disease-specific survival was 99% at 5 years. Cases of head and neck DFSP were found to occur more frequently in males. There was no difference in incidence rates between White patients and Black patients. Age ≥ 60 years old, tumor size, and living location were the most significant predictors of overall survival. CONCLUSIONS: This analysis of DFSP of the head and neck demonstrates a downward trend in incidence, higher age-standardized incidence in males, and similar race-based incidences, which differs from data reported on DFSP of other anatomic locations.

Changes in aqueous and vitreous inflammatory cytokine levels in diabetic macular oedema: a systematic review and meta-analysis.

Diabetic macular oedema (DME) is considered a chronic inflammatory disease associated with aberrations in many intraocular cytokines. Studies assessing the role of these cytokines as biomarkers in the diagnosis and management of DME have demonstrated inconsistent findings. We quantitatively summarized data related to 116 candidate aqueous and vitreous inflammatory cytokines as biomarkers in DME. A systematic search without year limitation was performed up to 19 October 2020. Studies were included if they provided data on aqueous or vitreous cytokine concentrations in patients with DME. Effect sizes were generated as standardized mean differences (SMDs) of cytokine concentrations between patients with DME and controls. Data were extracted from 128 studies that included 4163 study eyes with DME and 1281 control eyes. Concentrations (standard mean difference, 95% confidence interval and p-value) of aqueous IL-6 (1.28, 0.57-2.00, p = 0.004), IL-8 (1.06, 0.74-1.39, p < 0.00001), MCP-1 (1.36, 0.57-2.16, p = 0.0008) and VEGF (1.31, 1.01-1.62, p < 0.00001) and vitreous VEGF (2.27, 1.55-2.99, p < 0.00001) were significantly higher in patients with DME (n = 4163) compared to healthy controls (n = 1281). No differences, failed sensitivity analyses or insufficient data were found between patients with DME and healthy controls for the concentrations of the remaining cytokines. This analysis implicates multiple cytokine biomarker candidates other than VEGF in DME and clarifies previously reported inconsistent associations. As the therapeutic options for DME expand to include multiple agents with multiple targets, it will be critical to manage the treatment burden with tailored therapy that optimizes outcomes and minimizes treatment burden. Intraocular cytokines have the promise of providing a robust individualized assessment of disease status and response to therapy. We have identified key candidate cytokines that may serve as biomarkers in individualized treatment algorithms.

Validating the use of a stereoscopic robotized teleophthalmic drone slit lamp.

OBJECTIVE: To validate the use of a mechanized remotely operated stereoscopic drone slit lamp (DSL) in assessing anterior segment pathology in ophthalmology patients compared with conventional slit lamp (CSL). METHODS: Patients were recruited from eye clinics at Hotel Dieu Hospital in Kingston, Ontario, Canada. Each patient was assessed by 2 examiners. Examiners consisted of ophthalmology residents and staff attendings. Each examiner assessed the anterior chamber (AC) depth, presence or absence of cells, and/or presence of flare of the patient first using the DSL, followed by CSL. Qualitative data were collected on the ability to assess corneal integrity, infiltrates, foreign bodies, epithelial defects, and conjunctival injection using the DSL. RESULTS: 48 eyes of 42 participants were examined using the DSL and CSL. No significant within-examiner differences in AC depth or cell were detected. There was substantial agreement between the DSL and CSL when assessing AC cell and flare (κ = 72.6 and κ = 60.4, respectively) and moderate agreement when assessing AC depth (κ = 42.5). The DSL compared with CSL had a sensitivity and specificity of 98.3% (95% confidence interval [CI] 94-100) and 100% (95% CI 98.7-100), respectively, for detecting AC cell. The DSL had sensitivity and specificity of 100% (95% CI 97.5-100) and 88.2% (95% CI 80.2-96.1), respectively, for detecting AC flare. CONCLUSIONS: There was substantial agreement between the DSL and CSL when assessing AC depth, cell, and flare. Sensitivity and specificity for assessing these findings ranged from 88.2% to 100%. This DSL provides excellent capability for examination of anterior segment pathology in live patients, performing similarly to a CSL.

The incidence of giant cell arteritis in Ontario, Canada.

OBJECTIVE: The incidence of giant cell arteritis (GCA) is insufficiently documented for Canada, but important to ascertain for public health planning. We estimate the incidence of biopsy-proven GCA (BPGCA) in Kingston, Ontario, and for the province of Ontario. METHOD: The number of cases of BPGCA was tabulated from retrospective chart review of all temporal artery biopsies (TABx) in Kingston, Ontario from 2011-15. The relevant population denominator was determined from the Canada census federal electoral district and the patient's postal code. The province-wide estimate for the incidence of BPGCA was calculated from provincial billing data of TABx from 2015-17, the Canada census for Ontario, and the expected positive yield of TABx. RESULTS: There were 35 subjects with BPGCA in the Kingston area over the 4-year period, from a population of 179 503 individuals 50 years of age or older (≥50 years). Ontario billing data identified 2404 patients who underwent TABx for suspected GCA over a 2-year period, from a population of 5 143 610 persons ≥50 years. Meta-analysis of 5 provincial TABx series suggested a 21% positive yield from TABx procedures (95% CI 0.18-0.24). The minimum cumulative incidence of BPGCA was 4.9 per 100 000 persons ≥50 years in Kingston, and 4.9 (95% CI 4.2-5.6) per 100 000 persons ≥50 years for Ontario as a whole. CONCLUSION: The estimated incidence of BPGCA in Ontario using 2 different estimation techniques was comparable, but low compared with other countries. The actual incidence of GCA in Ontario may be higher.

Multivariable prediction model for suspected giant cell arteritis: development and validation.

PURPOSE: To develop and validate a diagnostic prediction model for patients with suspected giant cell arteritis (GCA). METHODS: A retrospective review of records of consecutive adult patients undergoing temporal artery biopsy (TABx) for suspected GCA was conducted at seven university centers. The pathologic diagnosis was considered the final diagnosis. The predictor variables were age, gender, new onset headache, clinical temporal artery abnormality, jaw claudication, ischemic vision loss (VL), diplopia, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and platelet level. Multiple imputation was performed for missing data. Logistic regression was used to compare our models with the non-histologic American College of Rheumatology (ACR) GCA classification criteria. Internal validation was performed with 10-fold cross validation and bootstrap techniques. External validation was performed by geographic site. RESULTS: There were 530 complete TABx records: 397 were negative and 133 positive for GCA. Age, jaw claudication, VL, platelets, and log CRP were statistically significant predictors of positive TABx, whereas ESR, gender, headache, and temporal artery abnormality were not. The parsimonious model had a cross-validated bootstrap area under the receiver operating characteristic curve (AUROC) of 0.810 (95% CI =0.766-0.854), geographic external validation AUROC's in the range of 0.75-0.85, calibration pH-L of 0.812, sensitivity of 43.6%, and specificity of 95.2%, which outperformed the ACR criteria. CONCLUSION: Our prediction rule with calculator and nomogram aids in the triage of patients with suspected GCA and may decrease the need for TABx in select low-score at-risk subjects. However, misclassification remains a concern.