Michael K. Yoon

Purpose: This study’s purpose was to investigate the outcomes of immune checkpoint inhibitor (ICI) treatment of periocular cutaneous squamous cell carcinoma with orbital invasion. Methods: A retrospective review was performed of 19 patients with biopsy-confirmed, advanced periocular cutaneous squamous cell carcinoma with orbital invasion treated with ICIs at a single institution. Tumors of conjunctival, sinonasal, lacrimal sac, or indeterminate origin were excluded. Demographic and clinical features at presentation, duration (number of cycles) of ICI, and treatment response were analyzed. Results: Nineteen patients with periocular cutaneous squamous cell carcinoma with orbital invasion treated with ICI were identified. The median age at diagnosis was 74 (interquartile range, 67–78) years. Most patients were non-Hispanic White (95%, n=18) and male (68%, n=13). Eight patients (42%) had AJCC T4 disease. Median presenting logMAR visual acuity in the affected eye was 0.3 (0.2–0.8). Eighteen patients (95%) had orbital involvement, and 14 (74%) had perineural involvement. Thirteen (68%) were treated with cemiplimab and 5 (26%) with pembrolizumab, and 1 was transitioned to cemiplimab after progression on pembrolizumab. Patients received a median of 9 (interquartile range, 6–15) cycles. Nine patients (47%) had complete response, 6 (32%) had a partial response, 3 (16%) remained stable, and only 1 (6%) progressed on ICIs. No tumors recurred nor required exenteration by the end of the study period. The median follow-up time was 35 (interquartile range, 19–53) months. Conclusions: Immunotherapy demonstrated excellent, sustained clinical outcomes in periocular cutaneous squamous cell carcinoma with orbital invasion with low morbidity and mortality. A disease control rate of over 90% was achieved and sustained at a median follow-up of almost 3 years.

Histiocytic sarcoma is an extremely rare malignancy typically presenting in extranodal sites, including the gastrointestinal tract, lung, nasal cavity, and soft tissue. Primary presentation in the skin is an even rarer entity, with only one prior case reported in the eyelid skin. Herein, we describe a case of histiocytic sarcoma that presented in the eyelid of an 80-year-old man. The lesion presented as a rapidly growing left lower eyelid lesion over 4 months. Shave biopsy revealed histologic and immunophenotypic findings most consistent with a morphologically low-grade histiocytic sarcoma. Complete excision was performed with an additional 2 mm peripheral and deep margins, which were found to be clear of malignancy. Systemic work-up and whole body imaging showed no evidence of metastases or other associated malignancy. The decision was made to forego radiation and proceed with close observation every 3 months. Two years from presentation, the patient had no evidence of recurrence.

The rise of artificial intelligence has ushered in a wave of interest and research on its potential applications in ophthalmology, including oculoplastic surgery. This review summarizes current efforts to incorporate artificial intelligence in the management of various oculoplastic conditions, including eyelid neoplasms, blepharoptosis, thyroid eye disease, orbital fractures, orbital lesions, nasolacrimal duct obstruction, as well as patient education and counseling.

Immunoglobulin G4-related disease (IgG4-RD) comprises a spectrum of inflammatory conditions characterized by lymphoplasmacytic infiltration by IgG4+ plasma cells, resultant tumefactive lesions at various locations in the body, storiform fibrosis, and elevated serum IgG4 levels. IgG4-related ophthalmic disease (IgG4-ROD) can occur in isolation or in conjunction with systemic findings. The most common manifestations of IgG4-ROD are dacryoadenitis and eyelid edema, though patients may also have trigeminal nerve enlargement, extraocular muscle involvement, and other orbital soft tissue lesions and inflammation. Workup is multifactorial and should include full body imaging, serum IgG4 levels, and biopsy, which must include staining for IgG4+ plasma cells and may show hallmark storiform fibrosis and/or obliterative phlebitis. IgG4-ROD can be misdiagnosed due to potential clinical, radiographic, and histopathologic overlap with other orbital inflammatory conditions; however, the presence of infraorbital or supraorbital nerve enlargement on imaging, as well as biopsy with strong levels of IgG4 histochemical staining, can be helpful in distinguishing IgG4-ROD from other orbital inflammatory pathology. Steroids are typically first-line treatment, although given high rates of recurrence, patients may require concurrent or subsequent treatment with steroid-sparing agents, most commonly rituximab.

PurposeThe current study queries the American Academy of Ophthalmology (AAO) Intelligent Research in Sight (IRIS) registry for data on the epidemiology, work-up, and management patterns of autoimmune orbital inflammation.MethodsAnalysis and description of patient data from the IRIS registry between 2013 and 2019 reviewing patients with autoimmune or idiopathic orbital inflammation with filters based on International Classification of Disease (ICD) and Current Procedural Terminology (CPT) codes. Patients with thyroid eye disease, orbital cellulitis, and orbital abscess were excluded.Main Outcome MeasuresDemographic descriptions included gender, age, geographic region, and treatment. Sub-analysis was performed by assessing rates of imaging, biopsy, lab work-up, and diagnostic categories.ResultsIn a final cohort of 20,584 patients, the mean age of onset of orbital inflammation was 51.7 years; 67% female; and 63% Caucasian, 21% unknown, 12% Black, 2.6% Asian, and 1.5% other. Only 49 had imaging, 78 had laboratory work-up, and 1,411 had biopsy codes. Treatment results showed 166 patients receiving antibiotics, 224 patients receiving steroids, and 35 patients receiving both.ConclusionsThis study assessed the epidemiology, diagnostic patterns, and treatment patterns for orbital inflammation through the AAO IRIS registry. Practise patterns suggest a relatively low overall rate of imaging and laboratory studies compared to biopsies, although this certainly under-represents the actual number of imaging and laboratory studies and exemplifies the inherent imprecision of using a large database. However, the methodology of this study provides a framework of approaching the IRIS registry for oculoplastic research.

A 25-year-old woman with a history of acute lymphoblastic leukemia with Philadelphia chromosome (BCR 22q11.2-ABL1 (9q34)) presented with two months of progressively worsening swelling and pain of her left upper eyelid. Magnetic resonance imaging showed a heterogeneously enhancing mass lesion in the left lacrimal gland. An incisional biopsy was performed; histopathology showed atypical lymphocytic cells and flow cytometry revealed a similar immunophenotype to her previously diagnosed cancer. Her presentation was consistent with recurrence of acute lymphoblastic leukemia in the lacrimal gland. The lacrimal gland can rarely be involved in relapse of acute lymphoblastic leukemia.

Hematologic malignancies such as leukemia and lymphoma can frequently present in the orbit; however, involvement of the extraocular muscles is rare. The authors report two cases of systemic hematologic malignancy presenting with bilateral extraocular muscle enlargement and associated compressive optic neuropathy (CON). Both patients experienced clinical and radiographic improvement of ocular and systemic manifestations of disease with prompt initiation of targeted chemotherapy. These cases highlight the importance of including hematologic malignancy in the differential diagnosis of atypical bilateral extraocular muscle enlargement.

Purpose: To quantitatively evaluate safety profile for botulinum toxin A (BTX-A) injections among patients undergoing treatment for cosmetic indications is produced, with special attention to clinically relevant covariates and their relative impact on safety. Methods: A systematic literature search was performed using PubMed (1996-January 2020) and Embase (1947-January 2020) to identify all randomized controlled trials (RCTs) that reported safety data for patients receiving BTX-A for cosmetic indications compared to placebo. A meta-analysis was performed to determine pooled risk ratios (RR) for treatment-related adverse events (TRAEs) and for specific adverse events. Meta-regression and additional analyses were performed for significant and/or clinically relevant covariates. Results: Following the review of 8,690 studies, 32 RCTs involving 9,669 patients were included. The pooled RR of any TRAE occurring after BTX-A injection compared to placebo injection was 1.53 (95% CI, 1.33–1.77; p < 0.001). Statistically significant covariates included individual injection volume and total injection volume. The type of BTX-A formulation, treatment site, total BTX-A units, and BTX-A units per injection were not significant. Specific adverse events more likely to occur following BTX-A injection rather than placebo injection included eyelid/eyebrow malposition (RR 3.55; p < 0.001), facial paresis (RR 2.42; p = 0.316), and headache (RR 1.45; p = 0.003). Injection site reactions and injection site bruising occurred at similar rates in both groups. Conclusions: The overall safety profile of BTX-A is acceptable and consistent with previous publications. The authors’ additional analyses provide a relative comparison of the impact of various treatment parameters on safety.

A 13-year-old boy presented with 3 days of left-sided periorbital pain, swelling, mucoid discharge, and fever to 103°F, with onset 1 day after swimming in the ocean. Within 12 hours, he experienced rapid clinical deterioration with formation of a superomedial subperiosteal abscess and an epidural abscess with leptomeningitis despite treatment with broad-spectrum intravenous antibiotics. The patient underwent urgent left orbitotomy with abscess drainage and functional endoscopic sinus surgery. Intraoperative cultures grew Shewanella algae and Escherichia coli. The patient showed marked clinical improvement following surgical intervention and tailored antibiotic therapy. This is the first reported case of orbital abscess with acute bacterial rhinosinusitis due to infection with Shewanella algae.