Welk, Grant M.

Purpose To investigate the clinical features, treatments, and outcomes of patients with secondary orbital lymphoma. Major Findings In this cohort of 31 patients, 15 (48.4%) were female and 27 (87.1%) were white. Follicular lymphoma was the most common subtype (n = 13, 41.9%) and was more associated with unilateral disease compared to other subtypes (p = 0.01). Common presenting symptoms included a palpable mass (n = 17, 54.8%), eyelid swelling (n = 16, 51.6%), and ptosis (n = 12, 38.7%). Patients were most often diagnosed with Ann Arbor Stage IV lymphoma (n = 17, 54.8%) via incisional biopsy (n = 13, 41.9%). Radiation therapy (n = 9, 29.0%) was the most common initial treatment, and most patients received radiation (n = 19, 61.3%) and/or chemotherapy (n = 21, 67.7%). A majority of patients had final best corrected visual acuity (BCVA) within 1 line of initial BCVA (n = 19, 82.6%) and had no local recurrences (n = 21, 67.7%). However, complete remission was rare (n = 3, 9.7%), and systemic recurrence occurred in over half of patients (n = 18, 58.1%). Conclusions Follicular lymphoma was the predominant subtype of secondary orbital lymphoma in this cohort. Most patients with secondary orbital lymphoma presented with advanced disease where few achieved complete remission and more than half of the patients developed systemic recurrence despite majority retaining visual acuity.

Background: Eight Global Ophthalmology (GO) fellowship programs are available in the United States, and more than 30 U.S. GO fellows are now alumni. There are no standardized learning objectives for these programs, and fellows’ experiences vary widely between institutions. Purpose: To survey GO fellowship program directors in the United States to elucidate differences in learning objectives, duration of time abroad, funding sources, and other differences between GO fellowships. Methods: All program directors at all institutions with current GO fellowship programs in the United States were sent a 25-question questionnaire via email from October through December 2022. The questionnaire included distinct domains regarding the GO fellowship experience, care delivery, didactics, and best practices. Most questionnaire items were quantitative; 3 were qualitative. Responses to the quantitative and qualitative questions items were summarized descriptively. Results: All 10 program directors from the 8 GO fellowship programs that were current at the time of the survey participated. GO fellowship positions at most (88%) institutions were funded, in part, by the billing of care services provided by the fellows, with 50% of programs receiving supplemental funding from donations and 25% from nongovernmental organizations. Non-GO duties at the program home institutions constituted 29.5% of the time spent during the fellowships, on average. Policy advocacy occupies 2.3% of fellowship time, on average. Specific didactic topics covered in the GO programs and director perspectives on the pragmatic best practices for GO outreach varied considerably; however, inclusion of at least some public health topics and ethics appeared to be consistent among all of the programs. Conclusions: These findings provide insights into the landscape of GO fellowship programs in the United States, highlighting variations in funding, formal educational opportunities, and the allocation of time fellows spent working at the program home institution. Establishing standardized core competencies for fellows is recommended.

Seronegative neuromyelitis optica spectrum disorder (NMOSD) is a diagnostic challenge. This study aimed to characterize misapplication of the 2015 international criteria for seronegative NMOSD. We reviewed 35 consecutive seronegative NMOSD referrals to a tertiary center. Two (6%) met formal criteria, and 2 (6%) were clinically suspected of NMOSD without fulfilling radiologic supportive criteria. Alternative diagnoses were common including multiple sclerosis (37%), optic neuritis (14%), functional neurologic disorder (11%), and other (38%). Immunosuppression with rituximab was used in 3 of 4 patients with functional neurologic disorder. Misapplication of the 2015 criteria is common, and alternative explanations in seronegative NMOSD patients should be strongly considered.

Purpose To describe the clinical characteristics, histopathologic subtype distribution, treatment, and outcomes of patients with primary orbital lymphoma. Major Findings There were 126 biopsy-confirmed cases of primary orbital lymphoma, with 81 (64.3%) female and 110 (87.3%) white. The average age at diagnosis was 62.7 ± 13.9 years. There were 26 (20.6%) cases with bilateral involvement. The most frequent lymphoma subtype was MALT lymphoma (N = 76, 60.3%), followed by diffuse large B-cell lymphoma (N = 18, 14.3%) and follicular lymphoma (N = 12, 9.5%). Prognosis was generally favorable, as local recurrence occurred in only 14 (11.1%) patients and final best corrected visual acuity (BCVA) loss of >1 line was seen in 30 (28.0%) patients. Bilateral lymphoma and advanced stage (Ann Arbor Stage III or IV) at presentation were associated with increased risk of local recurrence ( p = 0.0025, p = 0.049). Improved disease-specific survival was associated with diagnosis at Stage I or II ( p = 0.013), MALT subtype ( p = 0.034), or lack of chemotherapy requirement as primary treatment ( p = 0.0073). Conclusions In this single center academic cohort, MALT lymphoma subtype was most common. Overall orbital recurrence frequency was low, and bilateral lymphoma and advanced stage were associated with increased risk of local recurrence. MALT lymphoma subtype was associated with improved disease-specific survival. Long-term follow-up showed that most patients retained good visual acuity in the affected eye(s).