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Purpose: To evaluate the effectiveness of cemiplimab, a Programmed-cell-death-1(PD-1) protein inhibitor, for the treatment of cutaneous periocular-locally-advanced squamous-cell-carcinoma (POLA-SCC) with orbital-invasion., Methods: Multicentre real-world retrospective study. Demographic and clinical data were collected and analysed for patients with biopsy-proven POLA-SCC(AJCC-T4) with orbital-invasion who were treated with cemiplimab at one of four tertiary medical centres in 2019-2022., Results: The cohort included 13 patients, 8 males and 5 females, of median age 76 years (IQR65-86). The median duration of treatment was 5.0months (IQR3.5-10.5) and the median follow-up time, 15.0 months (IQR10.5-30). The overall response rate was 69.2%. Complete response was documented in seven patients (53.8%), partial response in two (15.4%), stable disease in one (7.7%), and progressive disease in two (15.4%); in one patient (7.7%), response was not evaluable. Six complete responders (46.1% of the cohort) received no further treatment and did not have a recurrence during an average follow-up of 6.14 (±6.9) months from treatment cessation. None of the patients underwent orbital-exenteration. The majority of adverse events were mild (grade-1), except for a moderate increase in creatinine level (grade-2), severe bullous dermatitis (grade-3), and myocarditis (grade-5) in one patient each. Four patients (30.7%) died during the follow-up period, all of whom had an Eastern-Cooperative-Oncology-Group score of 4 at presentation., Conclusions: To our knowledge, this is the largest study to date on cemiplimab therapy for cutaneous POLA-SCC with orbital-invasion. Treatment was shown to be effective, with an overall response rate of 69.2%. Cemiplimab holds promise for the treatment of patients with tumours invading the orbit as it may alleviate the need for orbital exenteration., (© 2023. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Objective: To evaluate the sensitivity and specificity of the ABCD progression display for keratoconus progression., Methods: Data was collected from patients that underwent at least two Pentacam assessments 6 months apart. Sensitivity and specificity were calculated for the ABCD progression display. Progression was defined by criterion 1: change in two ABCD parameters above 80% confidence interval (CI) or criterion 2: change in one ABCD parameter above 95%CI. Receiver operating characteristic analysis compared the area under the curve (AUC) of all ABCD parameter combinations., Results: Thirty eyes were evaluated over a median time of 10.3 months. Progression by criterion 1 resulted in a sensitivity of 61.9% and specificity of 88.9%. Progression by criterion 2 resulted in higher sensitivity (80.9%) and specificity (100%). Pairwise comparisons of the ROC curves show that the AUC achieved by criterion 2 was significantly higher than criterion 1 (0.905 vs. 0.754, p = 0.0332). Evaluation of all ABCD combinations with a significant change of 80% or 95% CI did not show superiority over criterion 1 or 2 regarding progression detection. The D parameter had a very low AUC (0.5-0.556)., Conclusions: The ABCD progression display can assess keratoconus progression with high sensitivity and specificity, thus assisting the patients' decision-making process. The D parameter did not contribute to the sensitivity or specificity of this classification., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Objective: To review the academic benchmark of highly influential ophthalmologists listed in the ophthalmology '2020 Power List'., Methods: In this cross-sectional study, the academic profiles, achievements, and bibliometric profiles of all ophthalmologists listed in the 'Power List of 2020', regarded as the most influential figures in ophthalmology today, were analysed., Results: Ninety-five ophthalmologists were studied, after excluding 10 non-ophthalmologist figures that are also listed. Their mean age is 63 ± 11.7 years, with a strong male predominance (84.2%, n = 80 males, P < 0.001). All have a medical doctorate degree, and 31% (n = 29) have a Philosophy Doctor (PhD) degree. Fifty-three percent (n = 51) are graduates of medical schools in the United States (US). However, non-US ophthalmologists have a higher percentage of PhD degrees (41%, 18/44) vs. US ophthalmologists (22%, 11/51, P = 0.069), and also a longer duration of post-residency training (5.8 ± 3.1 vs. 1.8 ± 0.9 years, P < 0.001). The most common subspecialty was cataract and anterior segment surgery (42%, n = 40). The mean and standard deviation of the total number of papers published by ophthalmologists were 307.4 ± 226.3, with a mean citation record of 11,835.7 ± 13,330.5, and a mean h-index of 46.9 ± 27.9., Conclusions: The ophthalmologists listed on the 'Power List of 2020' are leaders with high accomplishments and an established interest in research evidenced by a high record of publications and an exceptional bibliometric profile. The list contains more US figures with a gender disparity, demonstrating a greater difficulty for international ophthalmologists, especially women, in achieving this high level of recognition., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Purpose: To establish a model to predict treatment outcome of periocular locally advanced basal cell carcinoma (POLA BCC) based on initial response to treatment with vismodegib (Erivedge TM ), a sonic hedgehog inhibitor., Design: Subgroup analysis of data from the STEVIE study database., Methods: Analysis of medical history, treatment protocol, and treatment outcome of POLA BCC tumours in a STEVIE study population of 244 POLA BCC patients treated with ≥1 dose of vismodegib., Results: A predictive model for complete response (CR) was established based on the initial treatment response. A cutoff value of 20% reduction in tumour size at 3 months of treatment identified the patients with a high probability (82.76%) to achieve CR. A second cutoff value of 67.7% reduction in tumour size at 6 months of treatment improved the prediction to a 95.42% probability of a CR outcome., Conclusions: A treatment model was constructed based on the prediction of a CR outcome and the initial response to vismodegib treatment at 3 and 6 months. The study result provide significant new insights can facilitate decision-making on treatment management according to tumour response in patients with POLA BCC., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Objectives: To evaluate the effect of locally advanced periocular basal cell carcinoma (POLA-BCC) on health-related quality of life (HRQoL) and the benefit of vismodegib treatment among participants in the Safety Events in Vismodegib (STEVIE) trial between 2011 and 2017., Methods: The STEVIE trial was conducted in patients with BCC (all anatomic locations) who were treated with vismodegib in 28-day cycles. Patients completed the Skindex-16, a validated questionnaire for the analysis symptoms, emotions, and functioning, at baseline, on day 1 of cycle 2, on day 1 of cycle 7, and at the end-of-study visit. For the present study, data mining techniques were used to construct an ophthalmic database of the STEVIE study. Skindex-16 scores were compared among patients with POLA-BCC between baseline and follow-up and between patients with POLA-BCC and patients with locally advanced BCC on other sites of the head and face (controls)., Results: The cohort included 169 patients with POLA-BCC and 428 patients with non-periocular head BCC. Patients with POLA-BCC had a significantly worse overall functioning score at baseline than controls (p = 0.038) and a lower score specifically in activities of daily living (p = 0.001). At the last follow-up, patients with POLA-BCC showed significant improvement in scores for functioning (100%), symptoms (100%), and emotions (75%) relative to baseline., Conclusions: Secondary analysis of the results of the STEVIE trial showed that the HRQoL of patients with POLA-BCC is significantly impaired and can be greatly improved with vismodegib treatment., (© 2021. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)