Changes in retinal nerve fiber layer, ganglion cell complex, and ganglion cell layer thickness in thyroid eye disease: A systematic review.

Thyroid eye disease (TED) is characterized by progressive orbital inflammation. In severe cases, it can lead to dysthyroid optic neuropathy (DON), involving death of retinal ganglion cells and permanent vision loss. Imaging with optical coherence tomography (OCT) has enabled noninvasive examination of the structural impact of the disease, including of the retinal nerve fiber layer (RNFL), the ganglion cell layer (GCL), and the inner plexiform layer (IPL). In this study, we systematically review 20 studies (897 patients, 1430 eyes) to evaluate changes in retinal thickness in patients with TED, stratified by severity. Most studies found a decrease in thickness in peripapillary RNFL (pRNFL), non-pRNFL, combined RNFL/GCL/IPL, or combined GCL/IPL when comparing patients with DON to healthy controls or to patients without DON. However, results were equivocal regarding differences between non-DON TED patients and controls. In addition, several studies reported a postoperative decrease in pRNFL thickness after orbital decompression surgery. Future studies examining OCT changes of retinal thickness along with disease progression are needed to assess the potential of OCT in diagnosis and management of patients with TED.

The Impact of Primary Tumor Site on Survival in Mycosis Fungoides.

Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma (CTCL), but little is known about the influence of anatomic location of the primary disease site on overall survival (OS) and disease-specific survival (DSS). The purpose of this study was to examine the significance of primary tumor site on survival in MF. A search of the Surveillance, Epidemiology, and End Results (SEER) database was conducted for patients with a diagnosis of MF with a specified primary site from 2000 to 2019. Prognostic factors including demographic and tumor characteristics were examined using Cox regression models. Further research is needed to fully investigate primary disease site as a prognostic indicator, including a deeper dive into MF of all stages and subtypes.

Frozen Section Histoanatomy of the Peritarsal Eyelid.

BACKGROUND: Mohs surgery of eyelid skin cancers requires detailed knowledge of anatomy for precise surgery and accurate evaluation of histology. OBJECTIVE: To review the histology of the peritarsal eyelid using frozen sections as encountered intraoperatively by Mohs surgeons. METHODS: The authors review the literature describing the anatomy and histology of the peritarsal eyelid from the lens of a Mohs surgeon. Histology from select Mohs cases is used to frame the discussion of the microanatomy of this region. RESULTS: The peritarsal eyelids contain a unique mixture of skin, muscle, tarsus, glandular tissue, and conjunctiva. The histologic appearance of many of these structures differs from skin found outside of this anatomic region. Tumors of the eyelid and periocular region may mimic normal histologic structures found within the peritarsal eyelid. CONCLUSION: The peritarsal eyelids have unique anatomy and associated histologic structures. Knowledge of the detailed histoanatomy is required for confident execution of Mohs surgery in this anatomic region.

Maxillary Ameloblastoma with Local Recurrence, Orbital Invasion, and Systemic Metastases: A Case Report and Review of the Literature.

Introduction: Maxillary ameloblastoma is a rare, slow-growing odontogenic tumor that can recur after surgical excision, be locally aggressive, and rarely develop systemic metastases. We describe the course and management of a patient with recurrent maxillary ameloblastoma with orbital invasion and systemic metastases, the fourth case of its kind to be described in the literature. Case Presentation: A 50-year-old female presented with left hyperglobus. A diagnosis of maxillary ameloblastoma was made based on biopsy and neuroimaging with MRI and CT. Surgical management included partial maxillectomy with orbital floor reconstruction, given the orbital invasion. Three years later, left hyperglobus recurred, and the patient was found to have orbital recurrence and lung metastases on PET imaging. The lung and orbital lesions have responded well to chemoradiation therapy without surgical intervention. Conclusion: Maxillary ameloblastoma is a rare tumor that typically arises from odontogenic tissues. Though considered benign, they can recur and in the case of our patient, metastasize. Complete surgical excision with wide surgical margins is associated with a shorter average time to recurrence and a lower incidence of metastasis. Cases of metastasis are managed with chemotherapy with or without adjuvant radiotherapy. Precision medicine may play a role in managing this entity in the future, given the discovery of differing profiles of maxillary ameloblastoma compared to mandibular. Ophthalmologists should be aware of this tumor as it can invade the orbit, resulting in significant ocular morbidity and mortality.

Real-World Experience With Teprotumumab in Patients With Dysthyroid Optic Neuropathy.

BACKGROUND: Teprotumumab, an insulin-like growth factor I receptor inhibitory antibody, improved proptosis, diplopia, inflammatory signs/symptoms, and quality of life in patients with active thyroid eye disease (TED) in clinical trials. The trials excluded patients with dysthyroid optic neuropathy (DON). Recently, many case reports and case series have reported the successful use of teprotumumab to treat DON. Here, we review the data from published cases and our clinical experience in treating patients having DON with teprotumumab. METHODS: A literature search was conducted of patients with DON treated with teprotumumab from January 2020 through September 2022. Data from DON patients from the authors' (M.A.T. and C.A.B.) clinical practice were included. Primary outcome measure was mean (SD) improvements for visual acuity, color vision, and visual fields. Improvements in proptosis and clinical activity score (CAS) and diplopia were compared before and after teprotumumab administration. RESULTS: Ten observational studies/case reports were identified along with 2 patients in our practice. In all, there were 24 active TED patients with DON (37 eyes) who were treated with teprotumumab. Mean (SD) age was 66.5 (13.6) years and 13 (54%) were females, disease duration ranged from 2 months to >15 years. 22/24 patients had none, minimal improvement or progression of visual loss with intravenous/oral corticosteroids, orbital decompression (n = 9), and orbital radiation (n = 2). There were 2 patients who received teprotumumab as the only therapy. Overall, 88% (21/24) reported improvement in visual acuity after teprotumumab and in 75% (18/24), improvement in vision was observed after just 2 infusions of teprotumumab. Three eyes had decompression surgery in close proximity to teprotumumab infusions and were excluded from analyses. Mean (SD) improvement in visual acuity was 3.73 lines (SD 3.74), range 2-15 lines in 33 eyes. The mean (SD) improvement in the mean deviation on visual field testing in 15 eyes was 5.6 db (3.0 db). Mean (SD) improvement in proptosis was 4.37 mm (SD: 2.11) (20 patients, 32 eyes); and clinical activity score: mean reduction of 5.1 (1.3) for 18 patients. Teprotumumab was well tolerated in all but one patient. Adverse events reported included fatigue, dysgeusia, hearing loss, nausea, hyperglycemia, and muscle spasms. CONCLUSIONS: Teprotumumab is an effective treatment for DON in our experience and in published cases in whom treatment with steroids, surgery, or orbital radiation was unsuccessful.

Teprotumumab-Related Adverse Events in Thyroid Eye Disease: A Multicenter Study.

PURPOSE: To assess the duration, incidence, reversibility, and severity of adverse events (AEs) in patients with thyroid eye disease (TED) treated with teprotumumab. DESIGN: Multicenter, retrospective, observational cohort study. PARTICIPANTS: Patients with TED of all stages and activity levels treated with at least 4 infusions of teprotumumab. METHODS: Patients were treated with teprotumumab between February 2020 and October 2022 at 6 tertiary centers. Adverse event metrics were recorded at each visit. MAIN OUTCOME MEASURES: The primary outcomes measure was AE incidence and onset. Secondary outcome measures included AE severity, AE reversibility, AE duration, proptosis response, clinical activity score (CAS) reduction, and Gorman diplopia score improvement. RESULTS: The study evaluated 131 patients. Proptosis improved by 2 mm or more in 77% of patients (101/131), with average proptosis improvement of 3.0 ± 2.1 mm and average CAS reduction of 3.2 points. Gorman diplopia score improved by at least 1 point for 50% of patients (36/72) with baseline diplopia. Adverse events occurred in 81.7% of patients (107/131). Patients experienced a median of 4 AEs. Most AEs were mild (74.0% [97/131]), 28.2% (37/131) were moderate, and 8.4% (11/131) were severe. Mean interval AE onset was 7.9 weeks after the first infusion. Mean resolved AE duration was 17.6 weeks. Forty-six percent of patients (60/131) demonstrated at least 1 persistent AE at last follow-up. Mean follow-up was 70.2 ± 38.5 weeks after the first infusion. The most common type of AEs was musculoskeletal (58.0% [76/131]), followed by gastrointestinal (38.2% [50/131]), skin (38.2% [50/131]), ear and labyrinth (30.5% [40/131]), nervous system (20.6% [27/131]), metabolic (15.3% [20/131]), and reproductive system (12.2% [16/131]). Sixteen patients (12.2%) discontinued therapy because of AEs, including hearing loss (n = 4), inflammatory bowel disease flare (n = 2), hyperglycemia (n = 1), muscle spasms (n = 1), and multiple AEs (n = 8). CONCLUSIONS: Adverse events are commonly reported while receiving teprotumumab treatment. Most are mild and reversible; however, serious AEs can occur and may warrant treatment cessation. Treating physicians should inform patients about AE risk, properly screen patients before treatment, monitor patients closely throughout therapy, and understand how to manage AEs should they develop. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Risk Factors for Chalazion Diagnosis and Subsequent Surgical Excision.

PURPOSE: To identify factors associated with chalazion diagnosis and surgical excision. METHODS: Patients with an incident chalazion diagnosis from 2002 to 2019 were compared 1:5 with matched controls. Multivariable logistic regression was performed to identify variables associated with diagnosis and surgical excision. RESULTS: Chalazion patients (n = 134,959) and controls (678,160) were analyzed. Risk factors for diagnosis included female sex, non-white race, northeast location, conditions affecting periocular skin and tear film (blepharitis, meibomian gland dysfunction, rosacea, pterygium), non-ocular inflammatory conditions (gastritis, inflammatory bowel disease, sarcoidosis, seborrheic dermatitis, Graves' disease), and smoking (p < .001 for all comparisons). Thirteen percent of patients with chalazion underwent subsequent surgical excision. Diabetes and systemic sclerosis diagnoses decreased odds of diagnosis (p < .001). Male sex, rosacea diagnosis, Black and Hispanic race, antibiotic use, and doxycycline use increased odds of surgery (p < .001). CONCLUSION: Female sex, non-white race, conditions affecting periocular skin and the tear film, several non-ocular inflammatory conditions, and smoking were risk factors for chalazion diagnosis. Male sex, rosacea diagnosis, Black and Hispanic race, antibiotic use, and doxycycline use were risk factors for surgical intervention for chalazion. Our results prompt further study of these variables and their relationship to chalazion diagnosis to understand physiology and improve clinical outcomes. Furthermore, the results of this study suggest early recognition and treatment of concomitant rosacea may serve an important role in the management of chalazion and in the prevention of surgical intervention.

The treatment of refractory periorbital dermatitis.

Purpose: Refractory periorbital dermatitis has a chronic course with exacerbations leading to discomfort and cosmetic issues, yet characterization of treatment options is limited. Aims: The objective was to present comprehensive demographic data and medical management of a series of patients with refractory periorbital dermatitis. Settings and Design: Retrospective review identified patients treated at a single institution from January 2010 to August 2020. Methods: Descriptive analyses were performed. Demographic data and treatment history were reviewed and data including medication, use, date of use and discontinued use, reason for discontinuation (if applicable), refractory status, formulation, concentration, and dose frequency were extracted. Statistical Analysis Used: Descriptive analyses. Results: Forty-five patients were included. The average age at first diagnosis was 60.3 years (sd 14.9). 82.2% were women and 84.4% identified as Caucasian. Triamcinolone cream was most frequently used followed by tobramycin-dexamethasone, tacrolimus, and neomycin-polymyxin-dexamethasone. Less than 30% of patients on triamcinolone were refractory. 13.3% of patients used topical hydrocortisone, with over 80% of these patients experiencing refractory episodes of persistent irritation and erythema. Most patients were refractory during initial use or the first recurrence of periorbital dermatitis flare. Conclusions: By better characterizing the diverse treatment regimens in a unique subset of refractory patients, we hope to better inform potential courses of medical management for periorbital dermatitis.

Acute diplopia after glabellar hyaluronic acid filler injection.

Purpose: Blindness is a well-known complication of filler injection in the glabellar region. Acute diplopia from filler injection without vision loss is a rare complication that typically results in clinical ophthalmoplegia which can have permanent sequelae. Here, we describe a patient who presented with acute diplopia with grossly intact full extraocular motility after glabella hyaluronic acid filler injection that resolved after 1 month. Observations: A previously healthy 43-year-old woman underwent her first hyaluronic acid injection in the glabella and developed immediate binocular double vision with severe pain and skin mottling above her right eyebrow and central forehead. Hyaluronidase injections, nitroglycerin paste, and aspirin were immediately administered. On exam, there was significant skin mottling over the glabella, extending to the forehead and nose with a small incomitant horizontal and vertical misalignment. No changes to her vision were observed and extraocular motility was grossly full. The rest of her exam was unremarkable. Over the course of the following month, the patient's diplopia resolved, but she developed skin necrosis and scarring. Conclusions: Importance: Proper knowledge of facial and periocular anatomy is critical for practitioners to safely perform filler injections and manage potential complications. Patients should be counseled about the potential rare risks of these elective procedures.

Cavernous sinus thrombosis with bilateral orbital vein involvement and diffuse ischemic retinopathy / Trombose do seio cavernoso com envolvimento bilateral das veias orbitais e retinopatia isquêmica difusa

ABSTRACT A 53-year-old man with a 3-day history of periorbital swelling and vision loss in the left eye was found to have septic cavernous sinus thrombosis with bilateral orbital vein involvement causing congestive orbitopathy. He was treated with an emergent canthotomy and cantholysis, intraocular pressure-lowering drops, antibiotics, anticoagulation, and serial examinations. Optical coherence tomography ultimately revealed diffuse ischemic destruction of both layers of the retina, which suggested occlusion of the ophthalmic artery or the short posterior ciliary arteries and central retinal artery without intracavernous internal carotid artery involvement. The patient remained without light perception in the left eye after treatment.

RESUMO Um homem de 53 anos, com história de 3 dias de edema periorbital e perda de visão no olho esquerdo, apresentou trombose séptica do seio cavernoso com envolvimento bilateral das veias orbitais, causando uma orbitopatia congestiva. O paciente foi tratado com uma cantotomia e cantólise de emergência, colírios para redução da pressão intraocular, antibióticos, anticoagulantes e exames seriados. A tomografia de coerência óptica finalmente demonstrou destruição isquêmica difusa de ambas as camadas da retina, sugerindo uma oclusão da artéria oftálmica ou das artérias ciliares posteriores curtas e da artéria retiniana central, com ausência de envolvimento do segmento intracavernoso da artéria carótida interna. O paciente permaneceu sem percepção luminosa no olho esquerdo.

Mohs Micrographic Surgery for Facial Merkel Cell Carcinoma.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare malignant cutaneous tumor with frequent metastases. They often appear in the face where cosmetic and functional outcome is critical. Mohs micrographic surgery (MMS) is a controlled intervention that optimizes negative margins without sacrificing tissue. OBJECTIVE: A comprehensive assessment of outcomes of MMS-treated facial MCC will help guide clinicians in surgical and medical management. METHODS & MATERIALS: Retrospective review identified facial MCC cases treated with MMS at a single institution from January 2005 to August 2020. Tumor characteristics and outcomes were recorded and descriptive and predictive analyses were performed. RESULTS: 34 cases were reviewed with a mean followup of 34.4 months. The most common sites were the forehead, cheek-jaw region, and nasal ala. 2 (5.9%) patients had local recurrence by a mean of 4.3 months. No documented variables were significantly associated with local recurrence. 8 (23.5%) patients had progression to metastasis by a mean of 9.4 months. Younger age at biopsy and surgery, male sex, and intraoperative detection of in-transit disease were significantly associated with progression to metastasis. CONCLUSIONS: In summary, the tissue-sparing approach of MMS may be beneficial for MCC in cosmetically and functionally sensitive facial locations as it preserves tissue without compromising outcomes.

LGBTQ+ Identity and Ophthalmologist Burnout.

PURPOSE: To evaluate lesbian, gay, bisexual, transgender, questioning, and other sexual/gender minority (LGBTQ+) orientation as a burnout risk factor among an international ophthalmologist cohort. METHODS: An anonymous, cross-sectional electronic survey was distributed via an Internet platform to characterize the relationship among demographic factors, including LGBTQ+ orientation, and burnout as measured by the Copenhagen Burnout Inventory (CBI). Univariable data analysis (linear) by sexual orientation was performed and variables with an association with a P value of <0.15 in univariable analysis were included in the multiple linear regression modeling. RESULTS: A total of 403 ophthalmologists participated in the survey. The majority self-identified as "White" (69.2%), were from North America (72.0% United States, 18.6% Canada) and were evenly distributed between age of 30 and 65 years. Overall, 13.2% of participants identified as LGBTQ+ and 98.2% as cisgender. Approximately 12% had witnessed or experienced LGBTQ+-related workplace discrimination or harassment. The personal and work-related burnout scores and confidence limits of persons identified as LGBTQ+ were higher and nonoverlapping compared with those reported as non-LGBTQ+. Multivariable analysis identified significant risk factors for higher personal and work-related burnout scores: LGBTQ+ (11.8 and 11.1, P = .0005 and .0023), female gender (5.36 and 4.83, P = .0153 and .0434), older age (19.1 and 19.2, P = .0173 and .0273). and caretaker stress (6.42 and 5.97, P = .0085 and .0239). CONCLUSIONS: LGBTQ+ orientation is a burnout risk factor among ophthalmologists, and LGBTQ+ workplace discrimination may be a contributing factor. Support from ophthalmology organizations to address LGBTQ+-, gender-, and age-related workplace discrimination may decrease burnout. NOTE: Publication of this article is sponsored by the American Ophthalmological Society.

Systemic follicular lymphoma in a patient with a history of thyroid eye disease: a case report.

The most common cause of both unilateral and bilateral proptosis in adults is thyroid eye disease (TED), and the diagnosis in typical cases is made without imaging. However, many other orbital diseases, including inflammatory, infectious, and neoplastic processes, can mimic the symptoms of thyroid eye disease, highlighting the importance of imaging and biopsy in challenging cases. There are limited reports in the literature of orbital lymphoma being diagnosed in patients with a history of thyroid eye disease. Here, we present the case of a patient with long-standing TED who developed new asymmetric proptosis and was subsequently diagnosed with systemic follicular lymphoma.

Cavernous sinus thrombosis with bilateral orbital vein involvement and diffuse ischemic retinopathy.

A 53-year-old man with a 3-day history of periorbital swelling and vision loss in the left eye was found to have septic cavernous sinus thrombosis with bilateral orbital vein involvement causing congestive orbitopathy. He was treated with an emergent canthotomy and cantholysis, intraocular pressure-lowering drops, antibiotics, anticoagulation, and serial examinations. Optical coherence tomography ultimately revealed diffuse ischemic destruction of both layers of the retina, which suggested occlusion of the ophthalmic artery or the short posterior ciliary arteries and central retinal artery without intracavernous internal carotid artery involvement. The patient remained without light perception in the left eye after treatment.

Long-Term Burden of Pain and Reduced Visual Acuity after Gunshot Wounds to the Orbit.

PURPOSE: To characterize the clinical presentation, surgical management, long-term complications and outcomes of Gunshot Wounds(GSW) to the orbit. METHODS: An extended case series with retrospective chart-review was conducted on all cases (1985-2020) of traumatic ocular injuries secondary to GSWs at an academic institution with a level 1 trauma center. Predictors included demographic information, clinical and radiologic examination findings, and surgical intervention at time of presentation. Outcomes included long-term lid malposition, visual acuity, pain, and secondary surgery. Descriptive statistics and tests of association were performed, including Fishers exact tests for categorical data, Kruskal-Wallis rank sum tests, analysis of variance, and, in the case of repeated measures, generalized estimating equations. RESULTS: 88 patients with GSW involving the orbit were included with average age of 32.6 years (sd = 15.7). Patients were 85.2% male, 75% African-American, 25.0% Caucasian, and 5.7% Hispanic. Median follow up was 43.3 months (4.6, 136.4). The injuries at presentation were 53.4% intracranial, 21.6% open globe, 80.7% orbital fracture, 89.8% lid laceration. Visual acuity did not significantly improve over time. Long-term complications included abnormal lid or globe position in 26.1% of patients, reduced visual acuity in 55.2%, and persistent pain in 50.6%. CONCLUSION: To our knowledge, this represents the first and largest clinical epidemiologic study with insight into the clinical presentation, surgical intervention, and long-term outcomes of GSW to the orbit. The results demonstrate a long-term burden of persistent pain and reduced visual acuity in patients who suffer from GSW to the globe and orbit. This study may guide patient management and communications.

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 choroidal thickness and choroidal vascularity index in thyroid eye disease: a systematic review.

PURPOSE: To evaluate the evidence for changes in choroidal thickness and choroidal vascularity index (CVI) in people with thyroid eye disease (TED), stratified by severity, as measured by optical coherence tomography (OCT). METHODS: Systematic review, including quality assessment, of published studies investigating choroidal thickness in TED. Outcomes of interest included CVI, subfoveal choroidal thickness, mean choroidal thickness, and peripheral choroidal thickness in four quadrants (superior, inferior, medial, lateral). RESULTS: Nineteen studies were included which contained measurements from 1067 eyes in 798 patients with TED. Most studies found an increased CVI and increased choroidal thickness in patients with TED compared to normal controls, especially in the subfoveal region. In addition, several studies reported an increased thickness in patients with active vs. inactive TED, although the results are equivocal. Finally, many studies reported associations between increased choroidal thickness and worsened clinical measurements of disease activity, such as Clinical Activity Score (CAS). CONCLUSIONS: This systematic review provided evidence for an association between increased CVI/choroidal thickness and TED, suggesting a potential for OCT-measured CVI/choroidal thickness as an objective clinical marker. Given heterogeneity, future studies are needed to assess the role of measuring the choroidal changes in diagnosis and management of TED.

Orbital apocrine hidrocystoma with ptosis.

Purpose: To describe a patient with orbital apocrine hidrocystoma presenting with ptosis and subsequent management. Observations: A 43-year-old woman presented to the oculoplastic surgery clinic with a left-sided ptosis and enlarging but painless mass in the sulcus of the left upper eyelid. Magnetic resonance imaging demonstrated a large, circumscribed T2 bright cystic lesion in the extraconal space. Surgical excision and histopathology confirmed a diagnosis of apocrine hidrocystoma. Conclusions: Although uncommon, ptosis may be a presenting symptom of an orbital apocrine hidrocystoma, which should be considered in the differential diagnosis for an extraconal cystic lesion. Apocrine hidrocystomas are benign tumors and are cured with surgical excision with rare recurrence.