Advancing ocular oncology care through teleophthalmology: a patient-centric perspective.

OBJECTIVE: Assessing patient satisfaction is an important component of understanding a program's efficacy and quality. The purpose of this study is to evaluate patient satisfaction with a teleophthalmology program tailored for ocular oncology that provides screening, remote assessment, care planning, and follow-up. STUDY DESIGN: Retrospective survey. METHODS: An adapted version of the Telehealth Satisfaction Scale, consisting of the 8 questions plus 5 additional questions specific to the teleophthalmology program, was used to survey patients. The teleophthalmology program involves diagnostic imaging, remote physician assessments, and follow-up phone calls from health care team members to deliver results to patients. For reporting, the Consensus-Based Checklist for Reporting of Survey Studies was used. T-tests explored satisfaction differences across demographics and teleophthalmology visits. RESULTS: Among the 95 patients contacted, 91 agreed to participate in the survey (96% response rate). Participants exhibited high overall satisfaction with the teleophthalmology program, reflected in a mean Telehealth Satisfaction Scale score of 28.88 (SD = 3.35 of 32). The median number of teleophthalmology visits completed was 4.0 (range of 1-8 appointments). No significant differences in satisfaction were found on the basis of age (P = 0.56), urban or rural residence (P = 0.17), or the number of telemedicine appointments (P = 0.51). CONCLUSIONS: This analysis displays high satisfaction levels among patients within an ocular oncology clinic participating in the current telemedicine program, irrespective of age, geography, or number of teleophthalmology visits. Participants reported having their health care needs met with minimal clinical encounters due to remote consultations and telephone communications.

Artificial intelligence in the classification and segmentation of fundus images with choroidal nevi.

OBJECTIVE: The purpose of this study is to summarize the results from 3 experimental studies into the use of artificial intelligence to classify and segment colour fundus images with choroidal nevi. STUDY DESIGN: This study is based on a secondary analysis of colour fundus images taken of patients receiving usual clinical care from the Alberta Ocular Brachytherapy Program. METHODS: High-resolution colour fundus images were labeled by experienced ocular oncologists. In experimental study 1, four pre-trained models (ResNet 50, VGG-19, VGG-16, and AlexNet) were evaluated for their ability to classify images based on the presence of choroidal nevi. In experimental study 2, the performance of 3 patch-based models to classify images based on the presence of choroidal nevi were compared. In experimental study 3, four convolutional neural network models were developed to segment the images. In experimental studies 1 and 2, performance was measured using accuracy, precision, recall, F1 score, and AUC. In experimental study 3, IoU and Dice measures were used to evaluate performance. RESULTS: A total of 591 labelled colour fundus images were used for analysis. In experimental study 1, VGG-16 showed the best accuracy, AUC, and recall, but lower precision in classifying images. In experimental study 2, the patched approached enhanced with artifact and contrast outperformed the others in classifying images. In experimental study 3, a voting-based Ensemble model excelled in segmenting the part of images with nevi. CONCLUSIONS: It is feasible to train AI models to identify choroidal nevi in colour fundus images.

15-Gene Expression Profile and PRAME as Integrated Prognostic Test for Uveal Melanoma: First Report of Collaborative Ocular Oncology Group Study No. 2 (COOG2.1).

PURPOSEValidated and accurate prognostic testing is critical for precision medicine in uveal melanoma (UM). Our aims were to (1) prospectively validate an integrated prognostic classifier combining a 15-gene expression profile (15-GEP) and PRAME RNA expression and (2) identify clinical variables that enhance the prognostic accuracy of the 15-GEP/PRAME classifier.MATERIALS AND METHODSThis study included 1,577 patients with UM of the choroid and/or ciliary body who were enrolled in the Collaborative Ocular Oncology Group Study Number 2 (COOG2) and prospectively monitored across 26 North American centers. Test results for 15-GEP (class 1 or class 2) and PRAME expression status (negative or positive) were available for all patients. The primary end point was metastasis-free survival (MFS).RESULTS15-GEP was class 1 in 1,082 (68.6%) and class 2 in 495 (31.4%) patients. PRAME status was negative in 1,106 (70.1%) and positive in 471 (29.9%) patients. Five-year MFS was 95.6% (95% CI, 93.9 to 97.4) for class 1/PRAME(-), 80.6% (95% CI, 73.9 to 87.9) for class 1/PRAME(+), 58.3% (95% CI, 51.1 to 66.4) for class 2/PRAME(-), and 44.8% (95% CI, 37.9 to 52.8) for class 2/PRAME(+). By multivariable Cox proportional hazards analysis, 15-GEP was the most important independent predictor of MFS (hazard ratio [HR], 5.95 [95% CI, 4.43 to 7.99]; P < .001), followed by PRAME status (HR, 1.82 [95% CI, 1.42 to 2.33]; P < .001). The only clinical variable demonstrating additional prognostic value was tumor diameter.CONCLUSIONIn the largest prospective multicenter prognostic biomarker study performed to date in UM to our knowledge, the COOG2 study validated the superior prognostic accuracy of the integrated 15-GEP/PRAME classifier over 15-GEP alone and clinical prognostic variables. Tumor diameter was found to be the only clinical variable to provide additional prognostic information. This prognostic classifier provides an advanced resource for risk-adjusted metastatic surveillance and adjuvant trial stratification in patients with UM.

The Eye-Opening Truth About Private Surgical Facilities in Canada.

This paper examines the contentious issue of using contracted surgical facilities (CSFs) for scheduled eye surgeries within Canada's publicly funded healthcare system. Despite the debate over the use of CSFs, there is a stark lack of Canadian-focused empirical evidence to guide policy decisions. This paper uses the Organisation for Economic Co-operation and Development's healthcare system performance conceptual model - access, quality and cost/expenditures - as a framework to explore the debates surrounding CSFs. It highlights the mixed evidence from international studies and proposes recommendations for policy makers to ensure equitable access, maintain high-quality care and achieve cost-effectiveness. The paper underscores the necessity for informed policy making supported by robust empirical research, stakeholder engagement and continuous policy evaluation to address the challenges posed by the integration of CSFs into Canada's healthcare landscape.

Incidence of Strabismus Post-Plaque Brachytherapy in Patients With Uveal Melanoma.

PURPOSE: To determine the incidence and type of strabismus in patients with uveal melanoma treated with plaque brachytherapy. DESIGN: Multicenter, retrospective incidence estimation study. METHODS: A total of 438 eyes of 438 patients with uveal melanoma treated with plaque brachytherapy between October 2011 and May 2021. Intervention was Iodine 125, and Palladium 103 plaque brachytherapy. The variables reviewed included incidence of nonresolving strabismus post-plaque brachytherapy, type of strabismus developed, extraocular muscles operated, and modality of treatment received. RESULTS: A total of 438 patients underwent plaque brachytherapy treatment for uveal melanoma. Eleven patients developed strabismus post-plaque brachytherapy (2.5%, n = 11/438). Of these patients, 5 (1.1%, n = 5/438) developed strabismus immediately postoperation. Specifically, 2 patients (0.5%, n = 2/438) developed strabismus immediately postoperation due to slipped muscles, 2 patients (0.5%, n = 2/438) due to decompensated phorias, and 1 patient (0.5%, n = 1/438) due to a fibrotic muscle. Six patients (1.4%, n = 6/438) developed late-onset sensory strabismus. A total of 355 patients (81.1%, n = 355/438) had their extraocular muscles disinserted during surgery, with the lateral rectus being the most common, accounting for 45.4% (n = 161/355), followed by the superior rectus at 26.8% (n = 95/355). Strabismus surgery was the most common treatment modality, comprising 72.7% (n = 8/11) of patients. CONCLUSIONS: The incidence of strabismus after plaque brachytherapy treatment for uveal melanoma was low and primarily classified as late-onset sensory strabismus. Previous studies may underestimate the long-term incidence of strabismus after plaque brachytherapy by focusing primarily on strabismus present immediately postoperatively.

Primary Periocular Orofacial Granulomatosis: Case Series and Systematic Review.

PURPOSE: There is no known optimal treatment for primary periocular orofacial granulomatosis (PPOFG), a disorder that results in periocular edema. This case series and systematic review identifies management strategies and their reported improvement. METHODS: Systematic review and case series. PubMed and MEDLINE databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines to identify published cases of PPOFG. Cases were included when edema involved the periocular tissues and when a biopsy was interpreted to be PPOFG. Cases were excluded when edema did not involve the periocular tissues or when the edema was secondary to another process. The electronic medical records of the pathology department were studied to identify cases that were in keeping with PPOFG. The clinical charts were examined to confirm the diagnosis and provide a local case series. RESULTS: There are 38 published cases of PPOFG. An additional 9 cases were identified locally. These cases were combined and analyzed. Most PPOFG has eyelid swelling in isolation, without other facial swelling (36/47; 76.6%). It is most commonly a bilateral disease (30/47; 63.8%). Fissured tongue and facial nerve palsy occur, just as they do in other cases of orofacial granulomatosis. Treatment with surgical debulking or intralesional steroids resulted in high rates of symptomatic improvement of eyelid swelling, but recurrences were common. CONCLUSIONS: In light of no curative or highly successful treatment currently available, intralesional steroids and/or surgical debulking are therapies in the treatment of eyelid swelling associated with PPOFG that demonstrate reasonable short- and medium-term results. There is no established therapy that can offer disease remission or long-term symptom improvement.

Psychometric Results of a New Patient-Reported Outcome Measure for Uveal Melanoma Post-Brachytherapy Treatment: The PROM-UM.

The objective of this study was to evaluate the psychometric properties of a new patient-reported outcome instrument intended for use with patients who have undergone brachytherapy for uveal melanoma (PROM-UM). Classical test theory and item response theory were used to evaluate the performance of individual items and domains. A convenience sample of 439 participants who had undergone brachytherapy for uveal melanoma from one of three North American ocular oncology treatment centers were included in this cross-sectional study. Exploratory factor analysis identified three domains which were labelled "Symptom Impairment", "Worry", and "Discomfort". The acceptability of the instrument was supported by little missing data (range = 0.00-1.14%) and low maximum endorsement (range = 0.00-1.82%). Item-total (range = 0.68-0.85) and inter-item (range = 0.74-0.80) correlations indicated acceptable reliability. Discrimination and difficulty were assessed using item response theory. Items in all three domains indicated moderate to very high discrimination (range = 1.00-4.10). Two items in the Symptom Impairment domain were too difficult to measure. Response ranges in the other two domains demonstrated acceptable difficulty. These results from the study indicate that this new patient-reported outcome instrument can be used with patients treated with brachytherapy for uveal melanoma. Providers could use this instrument to help inform post-treatment management.

Outcomes of Second-Line Intravitreal Anti-VEGF Switch Therapy in Radiation Retinopathy Secondary to Uveal Melanoma: Moving from Bevacizumab to Aflibercept.

Introduction: Radiation retinopathy is a dose-dependent complication of the retina following exposure to ionizing radiation. The objective of this prospective case series is to determine the clinical efficacy of intravitreal aflibercept for radiation retinopathy secondary to radiotherapy for uveal melanoma in those that failed intravitreal bevacizumab treatment. Methods: A case series of 30 patients with a mean age of 57 ± 15 years with radiation retinopathy were enrolled. Visual acuity (VA) and central foveal thickness (CFT) responses to therapy were assessed with regression analyses at 1 month, 3 months, and 6 months following the switch to aflibercept. Results: Regression analyses showed a statistically significant reduction in CFT and improvements in VA following the switch to treatment by aflibercept at 1 month, 3 months, and 6 months. The mean CFT improved from 476 µm ± 170 to 386 µm ± 139 and the mean VA improved minimally from 20/115 ± 20/63 to 20/112 ± 20/54 over 6 months. After 6 months of aflibercept, 46% of patients displayed a CFT improvement of 100 µm or greater and 23% of patients showed improvement in VA of 1 line or better. Conclusion: This pilot study suggests that patients with radiation retinopathy who have failed monthly intravitreal bevacizumab may respond to aflibercept.

Management of Uveal Melanoma: Updated Cancer Care Alberta Clinical Practice Guideline.

OBJECTIVE: The purpose of this guideline update is to reassess and update recommendations in the prior guideline from 2016 on the appropriate management of patients with uveal melanoma. METHODS: In 2021, a multidisciplinary working group from the Provincial Cutaneous Tumour Team, Cancer Care Alberta, Alberta Health Services was convened to update the guideline. A comprehensive review of new research evidence in PubMed as well as new clinical practice guidelines from prominent oncology groups informed the update. An enhancement in methodology included adding levels of evidence and strength of recommendations. The updated guideline was circulated to all members of the Provincial Cutaneous Tumour Team for review and endorsement. RESULTS: New and modified recommendations address provider training requirements, diagnostic imaging for the detection of metastases, neo-adjuvant pre-enucleation radiotherapy, intravitreal anti-vascular endothelial growth factor agents for radiation retinopathy, genetic prognostic testing, surveillance following definitive local therapy, and systemic therapy for patients with metastatic uveal melanoma. DISCUSSION: The recommendations represent evidence-based standards of care agreed to by a large multidisciplinary group of healthcare professionals.

Sunlamp use is a risk factor for uveal melanoma: a meta-analysis.

OBJECTIVE: Advancements in the treatment of uveal melanoma have not improved survival; therefore, identifying modifiable risk factors is critical to improving outcomes. This study aims to investigate the association between sunlamp use and the development of uveal melanoma. DESIGN: This study is designed as a meta-analysis. METHODS: Literature was searched and reviewed through the MEDLINE (with both OVID and PubMed), EMBASE, MD Consult, and Web of Science databases. These databases were searched from 1966 to 2019 using the following keywords to identify articles examining risk factors for uveal melanoma: ultraviolet, sun, sunlight, uveal melanoma, eye cancer, eye melanoma, nevus, and risk factor. All articles were evaluated for inclusion based on methodology and data reporting association between sunlamp use and uveal melanoma. The Meta-analysis of Observational Studies in Epidemiology guidelines and the Newcastle-Ottawa Scale were used to assess data quality and validity. A random effects model was employed. RESULTS: A total of 5 studies, enrolling a total of 1753 uveal melanoma cases and 3399 controls were included in this meta-analysis. The results of this study showed a positive association between sunlamp use and uveal melanoma (odds ratio = 2.15; 95% confidence interval 1.27-3.64). Meta-regression of between study heterogeneity did not reveal a statistically significant association when publication year, site latitude, melanoma tissue location (specifically, inclusion of iris tumors), or control type (population versus clinic) were evaluated. CONCLUSION: This meta-analysis identified a statistically significant association between sunlamp use and uveal melanoma, supporting sunlamp use as a modifiable risk factor for uveal melanoma.

Optimising prediction of early metastasis-free survival in uveal melanoma using a four-category model incorporating gene expression profile and tumour size.

BACKGROUND: Largest basal diameter (LBD) appears to have independent prognostic value in uveal melanoma (UM). METHODS: All patients undergoing plaque brachytherapy or enucleation for UM involving the choroid and/or ciliary body between 2012 and 2019. RESULTS: A total of 348 patients with a mean age of 60±14 years were included and followed for a mean of 40±26 months (3.3±2.2 years). On multivariate analysis, LBD >12 mm remained a significant independent predictor of metastasis for both class 1 (HR 21.90; 95% CI 2.69 to 178.02; p=0.004) and class 2 (HR 2.45; 95% CI, 1.03 to 5.83; p=0.04) tumours. Four prognostic groups were created: group 1 (class 1, LBD <12 mm), group 2 (class 1, LBD ≥12 mm), group 3 (class 2, LBD <12 mm) and group 4 (class 2, LBD ≥12 mm). Life tables were used to calculate the 3-year and 5-year metastasis-free survival: group 1 (98 and 98%), group 2 (86 and 86%), group 3 (81 and 62%) and group 4 (54 and 47%). Compared with the reference category (group 1), the Cox proportional hazard model demonstrated a significant worsening of survival for each progressive category (group 2 (HR 21.59; p=0.004), group 3 (HR 47.12, p<0.001), and group 4 (HR 114.24; p<0.001)). In our dataset, the four-category Cox model performed poorer compared with the American Joint Committee on Cancer (AJCC) and gene expression profile (AJCC+GEP) in the Akaike's information criteria (AIC) (297 vs 291), fit better with the Bayesian information criteria (BIC) (309 vs 313) and performed similarly with the Harrel's C (0.86 (95% CI 0.80 to 0.91) vs 0.89 (0.84 to 0.94), respectively). CONCLUSIONS: Combination of GEP and LBD allows separation of patients into four easy-to-use prognostic groups and was similar to a model combining AJCC stage with GEP.

Open book technique for conjunctival tumors: a surgical approach to the fornix.

Surgical management of large tumors involving the conjunctival fornix can be challenging, as exposure and clear margins may be difficult to achieve. In this case series, we report our initial experience with the open book technique in 4 patients. Through a canthotomy and cantholysis, this surgical approach provides a wide surgical field and facilitates fornix reconstruction post large tumor excision. In our series, one patient had a lateral canthus dehiscence.

Rapid growth of primary uveal melanoma following intravitreal bevacizumab injection: a case report and review of the literature.

Uveal melanoma size is a significant predictor of tumor metastasis. Although the relationship between antivascular endothelial growth factors (VEGF) and uveal melanoma growth has been studied, results are paradoxical, and the relationship remains controversial. We report the case of a 65-year-old man who presented with elevated intraocular pressure in his right eye, neovascularization of his iris, and significant corneal edema, which obscured the view of the angle. Given his history of proliferative diabetic retinopathy, he was diagnosed with neovascular glaucoma and subsequently received an intravitreal injection of bevacizumab and underwent Ahmed valve insertion. This was complicated by postoperative hyphema. Two and a half months postoperatively, a mass involving the inferior iris and ciliary body became visible, and fine-needle aspiration biopsy confirmed uveal melanoma. Seven weeks after diagnosis, the tumor's largest basal diameter had increased from 2.51 mm to 18.0 mm, and apical height increased from 6.23 mm to 11.0 mm. His right eye was enucleated. Histopathological analysis showed discontinuous invasion next to the Ahmed valve. Tumor progression after injection raises the possibility that in some untreated uveal melanomas, accelerated growth may occur following exposure to anti-VEGF agents.

Automatic optimization of treatment dosimetry to improve visual outcomes in episcleral plaque brachytherapy.

PURPOSE: The aim of this study was to develop an automated dose optimization algorithm, powered by simulated annealing, for inverse planning of ocular plaque brachytherapy treatments, to improve tumor coverage and critical structure sparing for reduced treatment-related morbidity. METHODS AND MATERIALS: A simulated annealing based algorithm was built and evaluated by finding variable seed strengths that maximized dose uniformity across tumor base for model cases of various tumor heights and plaque sizes. This problem assessed its potential to find the minima in the energy function using differential loading. The algorithm was further developed to decrease doses to critical structures while maintaining desired tumor coverage. Doses to the optic disc and fovea were compared to those using uniform seed strengths for various model cases. Finally, 10 retrospective patient cases treated with uniform seed strengths were replanned with the developed algorithm to evaluate effectiveness for clinical application. RESULTS: The developed algorithm achieved dose reductions of up to 37.3% and 39.6% to the optic disc and fovea, respectively, compared to those using uniform seed strengths, depending on the tumor and plaque size used for the model cases. Applying the algorithm to 10 clinical scenarios resulted in dose reductions of 14% to the optic disc and fovea relative to clinical treatments performed with uniform seed strengths. CONCLUSIONS: The developed automatic dose optimization routine was able to achieve significant dose reductions to the critical structures relative to using uniformly loaded plaques both in the model and in the clinical cases.

The Alberta standardized orbital technique in the management of spheno-orbital meningiomas.

PURPOSE: To describe a standardized orbital resection technique and outcomes for spheno-orbital meningiomas with soft-tissue invasion of the orbit. METHODS: A retrospective case review of patients with spheno-orbital meningioma that underwent resection utilizing the Alberta Standardized Orbital Technique (ASOT) between 2008 and 2017 was performed. RESULTS: Twenty patients met the inclusion criteria. Fifteen females and five males, with an average age of 53.4 years (SD ± 13.1 years). Mean follow-up was 57.3 months (SD ± 29.5 months). Eight cases (40%) had attempted resection prior to referral. Based on pre-operative plan, patients were divided into two groups based on goal of resection. Of those with planned complete resection (Group I), 11/13 patients (84.6%) underwent complete excision, with no cases of orbital recurrence. Incomplete resection in two cases occurred because of unexpected involvement of critical intra-cranial structures. Thus, in total 9/20 patients (Group II and 2 from Group I) underwent subtotal resection. Of these incomplete resections, five cases of orbital recurrence were observed; four patients required adjuvant external beam radiotherapy (EBRT) and one patient underwent further debulking surgery. Orbital control was achieved in three of these recurrent cases. Complications reported were persistent postoperative diplopia (three cases/15%) and wound infection (one case/5%). Overall, stable orbital disease was obtained in 18 patients (90%). CONCLUSION: The ASOT demonstrated to be effective, achieving the pre-operative plan of complete resection in 11/13 cases (84.6%) with no recurrence in those with clear orbital margins.

A Predictive Model for Temporal Artery Biopsy in the Setting of Suspected Giant Cell Arteritis: A Validation Study.

PURPOSE: A previously published predictive model based on threshold parameters for erythrocyte sedimentation rate, c-reactive protein, and platelet count demonstrated that 40% of patients who underwent biopsy may not have required it. The current study was performed to evaluate the model's performance on an independent data set. METHODS: This is a retrospective consecutive series of patients undergoing temporal artery biopsy (TAB) in a single health region in Canada. The model was applied to a multicenter cohort of patients undergoing TAB by a variety of surgical services. A centralized pathological database serving multiple institutions and surgical services was used to identify patients undergoing TAB. RESULTS: Over a 7-year period, patients undergoing TAB were identified via a central pathological database. Those who had concurrent illnesses which would likely affect erythrocyte sedimentation rate, c-reactive protein, and platelet count, patients on steroids for >2 weeks by the time of biopsy, and those with missing serum markers were excluded. The previously developed model was applied to the 222 patients enrolled. The model correctly identified 29% of patients with a pretest probability of 0% for a positive biopsy and 9% with a pretest probability of 100%, suggesting that in total, 38% of patients could have avoided TAB. CONCLUSION: The results of this independent data set support the previously published predictive formula. Utilizing a simple, clinically applicable predictive model of the pretest probabilities, approximately 38% of TAB currently being performed may be avoided. The results suggest that evaluation with a prospective multicentre study would be appropriate.

Two Cases of Giant Melanocytoma (Hyperpigmented Magnocellular Nevus).

Melanocytoma or hyperpigmented magnocellular nevus is a variant of melanocytic nevus that is most commonly seen in the optic nerve, but has also been reported to occur in the iris, ciliary body, choroid, sclera, and conjunctiva. We present two cases of giant uveal melanocytoma with histopathology. The first case occurred in a 10-year-old girl who presented with decreased vision in the right eye and a mushroom-shaped pigmented choroidal lesion measuring 15.5 mm in apical height. The lesion was abutting the lens but not causing a cataract. This was diagnosed as a choroidal melanocytoma on open scleral window biopsy. The second case was in a 68-year-old lady, referred for a left nasal pigmented choroidal lesion measuring 8 mm in apical height and having a mushroom configuration. The lesion grew to 8.6 mm in height and was complicated by a vitreous hemorrhage and rhegmatogenous retinal detachment and was treated with iodine-125 plaque brachytherapy. Subsequently, the treated eye became a painful phthisical eye and was enucleated. Histopathology confirmed melanocytoma with extrascleral extension but without malignant transformation. Features of melanocytoma and other very large cases reported in the literature are discussed.