The Canadian Ophthalmology Society's adaptation of the Medically Necessary Time-sensitive Surgical Procedures triage and prioritization tool.

At the start of the coronavirus disease 2019 (COVID-19) pandemic, hospitals and ambulatory surgical centres significantly decreased elective surgical procedures to facilitate capacity for in-hospital beds, preserve personal protective equipment (PPE), preserve anesthetic medications and limit spread of infection. Non-COVID-19­related ophthalmic disease continues to affect vision, and it is anticipated that the backlog of elective surgeries will require months to years to resolve. The delivery of ophthalmic surgical care in a prioritized, systematic and transparent way is vital to manage the surgical backlog while minimizing vision loss and consequent disability in the Canadian population. The Canadian Ophthalmology Society (COS) has modified the published Medically Necessary, Time Sensitive (MeNTS) Procedures scoring system to be applicable to all subspecialties within ophthalmology. This case prioritization process integrates medical necessity, consideration of resource preservation with risk of COVID-19 exposure, and factors unique to eye care. It provides guidance to Canadian ophthalmologists to facilitate decision making in triaging elective procedures.

Guidelines for vismodegib in the management of periocular basal cell carcinoma.

OBJECTIVE: The management of advanced basal cell carcinoma (BCC) in the periocular region remains a clinical challenge. Vismodegib (ErivedgeTM) has been approved in 2013 by Health Canada for adult patients with "histologically confirmed metastatic BCC or locally advanced BCC inappropriate for surgery or radiation." An expert consensus was sought to create a standardised approach in the use of this novel treatment. METHODS: Fourteen practicing oculoplastic surgeons across Canada were involved in formulating and reviewing guidelines until consensus was reached. A consultancy meeting was followed by further ratification of guidelines over email. Two voting surveys were performed of the group to objectively assess agreement over each statement within the guidelines. Ratification continued until at least two-thirds of the group agreed on every guideline statement. RESULTS: The guidelines summarize 21 statements in a major and minor criteria format. A multidisciplinary team review is suggested for each patient with the involvement of recommended specialists. The internal survey revealed 100% agreement over 9 statements, 91.7% agreement over 8 statements, 83.3% agreement over 4 statements, and 2 statements had 66.7% and 58.7% agreement each. All statements with less than 91.7% agreement were surveyed again, and they were kept, modified, or removed on the basis of a consensus of over 66.7%. CONCLUSIONS: These guidelines serve to act as a framework for physicians considering vismodegib for the medical management of patients with advanced or metastatic periocular BCC. Future applications, including neoadjuvant uses of the drug, may become apparent through further research.

An In Vitro Model Assessing the Penetration of Hyaluronidase through Optic Nerve Dura for Management of Hyaluronic Acid Facial Filler Embolism.

BACKGROUND: Blindness from ophthalmic or central retinal artery embolism is one of the most devastating complications of cosmetic filler facial injections. A proposed therapy to mitigate visual loss is prompt retrobulbar injection of hyaluronidase into the retrobulbar space. Despite Zhu et al. showing a lack of evidence and very limited published literature for reversing visual loss with this intervention, it is still widely accepted as a treatment for filler-related emboli. The purpose of this study was to evaluate the penetration of hyaluronidase through optic nerve dura using an in vitro model. METHODS: At study conclusion, five 1-cm-long segments of fresh optic nerve were obtained and injected with highly crosslinked hyaluronic acid filler, then ligated on both ends in a watertight fashion. The sections were immersed in three concentrations of hyaluronidase solution for 24 hours. Histopathologic examination of the specimen was performed to assess the presence of filler. RESULTS: The optic nerve sections were 1.1 cm (range, 0.8 to 1.2 cm). Three were immersed in 20 ml of 1500 IU/ml hyaluronidase solution and two were immersed in saline as control. After 24 hours, there was a persistence of hyaluronic acid within the optic nerves. CONCLUSIONS: There is a lack of evidence for penetration of optic nerve sheath by hyaluronidase. This raises question about the effectiveness of retrobulbar injection of hyaluronidase in reversing filler-related blindness. Further studies are needed before this can be adopted as the treatment of choice. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Chemoreduction of Orbital Recurrence of Uveal Melanoma by Intra-Arterial Melphalan.

BACKGROUND/AIMS: The treatment of orbital melanoma poses a management challenge. This case explores the delivery of high-dose melphalan to an orbital recurrence of uveal melanoma via intra-arterial delivery of melphalan to the orbit. A 62-year-old man developed recurrent orbital disease 7 months after enucleation for a large uveal melanoma. He received 6 monthly intra-arterial infusions of melphalan to the orbit, ranging in dose from 20 to 30 mg per infusion. Following the last infusion, mild temporary erythema was noted on the forehead along the distribution of the supratrochlear artery. The orbital recurrence was reduced in size by 66% in the longest dimension as measured by magnetic resonance imaging (MRI). However, 9 months following intra-arterial melphalan, tumor regrowth was detected on MRI, and additional treatment options were pursued. CONCLUSION: This case demonstrates that intra-arterial melphalan can result in nonsustained tumor regression of recurrent orbital uveal melanoma. It suggests that local delivery of high-dose melphalan may be helpful as a neoadjuvant treatment for uveal melanoma, and future studies will be useful to confirm the value of this approach in additional cases of recurrent and possibly in primary uveal melanoma.

Nodular fasciitis: A rapidly enlarging destructive periorbital mass in an infant.

PURPOSE: To review the clinical and histopathological features of nodular fasciitis, a rare benign periorbital tumor that mimics orbital malignancy, by presenting a case involving an infant with marked orbital wall erosion requiring repair. OBSERVATIONS: A 9-month-old boy developed a rapidly growing periorbital mass concerning for a soft tissue malignancy. Computerized tomography (CT) scans showed bony erosion of the lateral orbital wall. Incisional biopsy revealed nodular fasciitis. USP6 gene rearrangement was negative. The tumor was completely excised and the underlying orbital wall defect was repaired with polydioxanone (PDS) plate. CONCLUSIONS AND IMPORTANCE: Nodular fasciitis is a benign periorbital tumor that presents like malignancies and warrants prompt investigations, especially in children. Orbital wall erosion is rare and can be repaired to yield good functional and cosmetic outcome.

Pathological Features and Clinical Course in Patients With Recurrent or Malignant Orbital Solitary Fibrous Tumor/Hemangiopericytoma.

PURPOSE: A risk assessment score for metastasis based on age, tumor size, and mitotic figures has been suggested for nonorbital solitary fibrous tumor (SFT)/hemangiopericytoma. The authors herein examine the clinicopathological features of recurrent and metastatic orbital SFT and evaluate the existing risk assessment score for orbital SFT. METHODS: The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried for patients with recurrent or malignant orbital hemangiopericytoma/SFT. The medical records were reviewed for clinical and pathologic findings, treatments, and outcomes. RESULTS: Eight patients from 3 institutions were identified with recurrent orbital hemangiopericytoma/SFT. Median age at diagnosis was 59 years, and 4 patients were women. The mean size of tumor was 2.1 ± 1.1 cm. All patients were initially treated with surgery and experienced local recurrence after a median of 4 (range 0.5-10) years. Five patients were treated with orbital radiation. Two patients also developed distant metastases and eventually died of their disease. Median Ki-67 was 5% (range 1-65%) and 5 mitotic figures/10 high-power fields (range 2-30). The previously described risk stratification model for nonorbital SFT did not correlate with the propensity to develop metastases in this cohort; however, both patients with distant metastasis had > 4 mitotic figures /10 high-power fields. CONCLUSIONS: In this cohort of recurrent orbital hemangiopericytoma/SFT, median time to recurrence was 4 years underscoring the importance of careful continued follow-up. The current risk stratification models have limited use for orbital lesions, mostly due to the fact that orbital SFTs are smaller than even the smallest size criteria in this risk assessment model.

Tumor Board Conferences for Multidisciplinary Skin Cancer Management: A Survey of US Cancer Centers.

Background: Tumor board conferences (TBCs) are used by oncologic specialists to review patient cases, exchange knowledge, and discuss options for cancer management. These multidisciplinary meetings are often a cornerstone of treatment at leading cancer centers and are required for accreditation by certain groups, such as the American College of Surgeons' Commission on Cancer. Little is known regarding skin cancer TBCs. The objective of this study was to characterize the structure, function, and impact of existing skin cancer TBCs in the United States. Methods: A cross-sectional online survey was administered to physician leaders of skin cancer TBCs at NCI-designated Comprehensive and Clinical Cancer Centers. Results: Of the 59 centers successfully contacted, 14 (24%) reported not having a conference where skin cancer cases were discussed, and 45 (76%) identified 53 physician leaders. A total of 38 physicians (72%) completed the survey. Half of the meeting leaders were medical and/or surgical oncologists, and dermatologists led one-third of meetings. TBCs had a moderate to significant impact on patient care according to 97% of respondents. All respondents indicated that the meetings enhanced communication among physicians and provided an opportunity for involved specialists and professionals to discuss cases. The most frequently cited barrier to organizing TBCs was determining a common available date and time for attendees (62%). The most common suggestion for improvement was to increase attendance, specialists, and/or motivation. Conclusions: Results showed overall consistency in meeting structure but variability in function, which may be a reflection of institutional resources and investment in the conference. Future directions include defining metrics to evaluate changes in diagnosis or management plan after tumor board discussion, attendance, clinical trial enrollment, and cost analysis. Results of this survey may aid other institutions striving to develop and refine skin cancer TBCs.

Prevalence of blindness and cataract surgical coverage in Narayani Zone, Nepal: a rapid assessment of avoidable blindness (RAAB) study.

BACKGROUND: The 1981 Nepal Blindness Survey first identified the Narayani Zone as one of the regions with the highest prevalence of blindness in the country. Subseuqently, a 2006 survey of the Rautahat District of the Narayani Zone found it to have the country's highest blindness prevalence. This study examines the impact on blind avoidable and treatable eye conditions in this region after significant increase in eye care services in the past decade. METHODS: The rapid assessment of avoidable blindness (RAAB) methodology was used with mobile data collection using the mRAAB smartphone app. Data analysis was done using the standard RAAB software. Based on the 2011 census, 100 clusters of 50 participants aged 50 years or older were randomly sampled proportional to population size. RESULTS: Of the 5000 participants surveyed, 4771 (95.4%) were examined. The age-adjusted and sex-adjusted prevalence of bilateral blindness, severe visual impairment (SVI) and moderate visual impairment (MVI) were 1.2% (95% CI 0.9% to 1.5%), 2.5% (95% CI 2.0% to -3.0%) and 13.2% (95% CI 11.8% to 14.5%), respectively. Cataract remains the primary cause of blindness and SVI despite cataract surgery coverage (CSC) of 91.5% for VA<3/60. Women still account for two-thirds of blindness. CONCLUSION: The prevalence of blindness in people over the age of 50 years has decreased from 6.9% in 2006 to 1.2%, a level in keeping with the national average; however, significant gender inequity persists. CSC has improved but continues to favour men.

Targeting the Hedgehog Pathway for Locally Advanced and Metastatic Basal Cell Carcinoma.

Basal cell caricnoma (BCC), the most common periocular magliancy, is treated with complete surgical excision. However, in patients not amenable to surgery or when surgical resection means loss of vital organs or disfiguring procedures due to locally advanced or metastatic disease, targeting the hedgehog pathway offers a novel treatment approach for such patients. Mutation in PTCH1 and SMO has been identified in patients with basal cell nevoid syndrome as well as in patients with sporadic BCC. Inhibition of SMO by vismodegib or sonidegib, the two sonic hedgehog inhibitor drugs approved by the Food and Drug Administration in the United States, has shown overall response rate for locally advanced and metastatic BCC around 50%. The most common side effects were muscle cramps, weight loss, fatigue, and lost appetite. Resistance to vismodegib has been attributed to mutation in SMO or activation of RAS/MAPK pathway. New research into dual inhibition aims to overcome this resistance and provide more lasting response.

Epidemiology and Prognosis of Primary Periocular Sweat Gland Carcinomas.

PURPOSE: To characterize the clinical presentation, management, and outcomes of patients with sweat gland carcinoma of the periocular region. To review the pathologic classification scheme, compare the clinical behavior between subtypes, and discuss treatment recommendations for periocular sweat gland carcinomas. METHODS: Retrospective study from 2 tertiary centers, which are part of the American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database. RESULTS: Twenty-two patients, of whom 13 (59%) were female, were included in the study. The median age was 69 years. The median follow-up time was 23 months. Twenty (91%) patients had lesions that were slow growing and were present for several months to years before diagnosis. Presentation was varied, and pathology included mucinous carcinoma (7; 32%), microcystic adnexal carcinoma (4; 18%), endocrine mucin-producing sweat gland carcinoma (4; 18%), eccrine carcinoma (2; 9%), apocrine carcinoma (2; 9%), poorly differential adnexal carcinoma (2; 9%), and hidroadenocarcinoma (1; 5%). Microcystic adnexal carcinoma tended to present with the largest tumor dimension (range: 25-32 mm) and mucinous carcinoma the smallest (range: 5-13 mm). Treatments included Mohs micrographic excision in 10 patients (45.5%), wide local excision in 10 patients (45.5%), and orbital exenteration in 2 patients (9%). No lesions ≤T2b (by AJCC 7th edition classification for eyelid carcinomas) had local recurrence or nodal metastasis. No patients had distant metastasis. CONCLUSIONS: Sweat gland carcinomas tend to grow slowly and be present for several months to years before diagnosis. Overall, microcystic adnexal carcinoma subtype showed more aggressive clinical behavior than mucinous subtypes. Eyelid carcinomas AJCC category T2b or less were associated with better outcomes.

Eye-sparing multidisciplinary approach for the management of lacrimal gland carcinoma.

BACKGROUND: We analyzed local control and early ocular toxicity after eye-sparing management of lacrimal gland carcinoma. METHODS: For consecutive patients with lacrimal gland carcinoma treated during 2007 to 2014, we reviewed tumor characteristics, treatment details, ocular toxic effects, and recurrence. RESULTS: Twenty patients, median age 55 years, were treated for lacrimal gland carcinoma during the study period; 11 had globe-sparing surgery. Seven patients had adenoid cystic carcinoma, 2 had carcinoma ex pleomorphic adenoma, and 1 each had high-grade and low-grade adenocarcinoma. Ten patients underwent postoperative radiotherapy, median 60 Gy (range, 52-64 Gy), 6 with concurrent chemotherapy. At a median of 30 months after radiation, all patients had dry eye syndrome, and 1 patient had severe corneal and conjunctival damage leading to enucleation. All 11 patients were disease free at last contact, median follow-up after surgery of 33 months. CONCLUSION: An eye-sparing approach with surgery followed by adjuvant radiotherapy or chemoradiotherapy is feasible for selected patients with lacrimal gland carcinoma and is associated with a reasonable locoregional control and ocular toxicity profile. © 2016 Wiley Periodicals, Inc. Head Neck 38:1258-1262, 2016.

Eyelid and ocular surface carcinoma: diagnosis and management.

Eyelid cancers account for 5% to 10% of all cutaneous malignancies. The incidence of eyelid cancer is approximately 15 cases per 100,000 individuals per year. Basal cell carcinoma is by far the most common cutaneous malignancy in the periocular area; other cutaneous malignancies that occur in this area include, in decreasing order of frequency, squamous cell carcinoma, sebaceous carcinoma, melanoma, and Merkel cell carcinoma. The most common treatment for eyelid carcinomas is surgical resection with frozen section examination for margin control, but exenteration may be needed when there is orbital invasion. Adjuvant radiotherapy may be needed in patients at high risk for local recurrence; sentinel lymph node biopsy may be considered in patients at high risk for lymph node metastasis. Primary or residual in situ disease of the conjunctiva can be treated with topical chemotherapy, such as mitomycin C, 5-fluorouracil, or interferon alpha-2 b. For patients with metastatic or locally advanced basal cell or squamous cell carcinoma not amenable to surgical excision or radiotherapy, targeted therapy against the hedgehog pathway (for basal cell carcinoma) or epidermal growth factor receptor (for squamous cell carcinoma) has been shown to be effective in preventing disease progression. Patients with eyelid and ocular surface malignancies need to be monitored with careful clinical examination for at least 5years after surgical treatment, and additional investigations may be warranted in some cases.

Cutaneous Eyelid Neoplasms as a Toxicity of Vemurafenib Therapy.

The discovery of BRAF mutation in ~50% of melanomas led to the development of small molecule BRAF inhibitors, including sorafenib, debrafenib, and vemurafenib. Clinical trials have shown these agents to be effective in treatment of metastatic and locally advanced melanoma, increasing overall and progression-free survival. However, some of the most common toxicities associated with BRAF inhibitor therapy include adverse skin events such as rashes, photosensitivity, hyperkeratosis, papillomas, keratoacanthomas, and squamous cell carcinomas. Here, the authors describe 3 patients who developed keratinocytic neoplasms on the eyelid, including invasive squamous carcinoma secondary to vemurafenib. Vigilant screening and a high index of suspicion for eyelid carcinomas are recommended in patients treated with vemurafenib.

The transeyelid midface lift.

The transeyelid approach to midface lift is an elegant approach for mild descent of malar soft tissue. The subciliary approach is the most commonly used and technically less challenging for surgeons experienced in facelift techniques. This technique in midface rejuvenation also has the advantage of ease of combining with other periocular and mid and upper face rejuvenation, such as blepharoplasty and forehead lift. Complication is rare with lid malposition, scaring, and temporary nerve function impairment being the most common.

Treatment Challenges with Benign Bone Tumors of the Orbit.

Benign mesenchymal tumors of the craniofacial complex present unique challenges for orbital surgeons because of their potential for orbital compartment syndrome, ocular morbidity, and facial disfigurement and because definitive surgical management may be associated with significant morbidity. While the precise classification of such lesions depends on radiologic as well as histologic evaluations and remains controversial, benign tumors involving the bony walls of the orbit share features of bony expansion, facial deformity, and the potential to cause significant orbital and ophthalmic morbidity. We herein present 2 cases of benign mesenchymal tumors with bony involvement in the orbitofacial region (1 juvenile ossifying fibroma and 1 central giant cell granuloma) and review the current management of similar benign fibro-osseous and reactive bone lesions of the orbit. These rare entities presented share common orbital and ophthalmic manifestations and remain without any effective definitive treatment options.

Targeting EGFR and sonic hedgehog pathways for locally advanced eyelid and periocular carcinomas.

For patients with metastatic or locally advanced eyelid and periocular carcinoma not amenable to surgical excision, targeted therapies have shown efficacy with better tolerability compared to cytotoxic chemotherapy. Overexpression of epithelial growth factor receptor was found in squamous cell carcinomas. Vismodegib targets the mutation in the hedgehog pathway identified in basal cell carcinoma and basal cell nevus syndrome. Targeted therapies provide a novel and potentially effective treatment alternative for patients with eyelid carcinoma not amendable for surgery, including those with metastatic, locally advanced disease, advanced age, and significant comorbidities. High cost, need for long-term treatment, and toxicity are relative limitations.

Number of excisions required to obtain clear surgical margins and prognostic value of AJCC T category for patients with eyelid melanoma.

AIMS: To determine the number of excisions needed to achieve clear margins and the prognostic value of the 7th edition of American Joint Committee on Cancer (AJCC) classification for eyelid melanoma. METHODS: Retrospective chart review of consecutive patients treated for eyelid melanoma from January 2006 through May 2013 by the senior author at a tertiary care cancer centre. RESULTS: Of the 64 patients (25 men and 39 women), clear surgical margins were achieved with a single excision in 38 patients (62%), 2 excisions in 21 patients (34%), and 3 excisions in 2 patients (3%). Need for repeat excision was not correlated with the size of the surgical margin (p=0.14) or AJCC TNM classification (p=0.15). Nodal disease at presentation was significantly associated with T category greater than T2b (p=0.0026) and shorter time to disease progression (p=0.007). Patients followed for a minimum of 1 year with T category greater than T2b had a significantly higher risk of nodal or distant metastasis (p=0.0061). CONCLUSIONS: More than a third of patients with eyelid melanoma required more than 1 excision to achieve clear margins, supporting delayed reconstruction for eyelid melanoma. Nodal metastasis at presentation was significantly correlated with AJCC T category and time to progression.