Seminars in Ophthalmology

effrey Jay Hurwitz is amongst the foremost prominent figures of Canadian ophthalmology. He was a visionary and an innovator. While several individuals have historically contributed to developing the lacrimal drainage sciences, Hurwitz has carved his unique place in history for posterity. Although several of his lacrimal works were unique, his two significant contributions were innovating newer techniques in lacrimal radiology and methods in clinical evaluation, which have stood the test of time. Hurwitz will always be remembered for expanding the boundaries of Dacryology and qualitatively enhancing research in lacrimal sciences.

A review of Silkiss and Sivak’s contributions reveals that they were astute clinicians and innovators. They have a distinct place among several individuals in the history of lacrimal sciences who have contributed to developing surgical techniques. Silkiss’ endeavor to improve lacrimal drainage surgeries using THC-YAG laser and one of the earliest movers towards a futuristic concept of NLD recanalization will remain engraved on the sands of time. Sivak’s reintroduction of the forgotten ultrasonic technology in ophthalmic plastic surgery, specifically in DCR surgery, will remain etched in lacrimal history.

Background: Long-term follow-up is essential for retinoblastoma survivors, as treatment-related sequelae can emerge years after therapy. Methods: This case series reviews 67 eyes treated with periocular and/or intravitreal chemotherapy from 2005 through 2020. Results: Periocular chemotherapy was associated with orbital fibrosis in 79.1% of cases, with risk increasing concomitantly with the number of injections. Cataracts developed in 19.4% of eyes, with 10 out of 13 requiring cataract extraction. Other complications included chronic conjunctivitis and limbal stem cell deficiency (3% each), while strabismus was observed in 29.9% of which 7.5% underwent strabismus surgery. Comparative analyses of periocular and anterior segment complications from other retrospective studies are included to contextualize our findings. Conclusions: Understanding these long-term sequelae is crucial for appropriately following and managing retinoblastoma survivors who were treated during this era. We highlight the importance of vigilant surveillance as treatment protocols continue to evolve.

Several of Jones’s innovations and observations, including those on CDCR, puncto-canaliculoplasty, and lacrimal pump, continue to hold high-value several decades after their initial description. The conjunctivodacryocystorhinostomy with Jones tubes is still performed worldwide for managing complex proximal canalicular obstruction, punctal agenesis, and select cases of post-DCR epiphora. His contributions have carved a special place for themselves in the medical world, and any history of lacrimal drainage science would be incomplete without him. Several generations of lacrimal surgeons have benefited from Jones’s work and innovations, and several more will continue to do so with gratitude. We take this opportunity to salute him for significantly impacting the landscape of lacrimal sciences and making this world better.

Purpose: Keratoconus is a bilateral asymmetrical degeneration in the cornea. It is associated with local thinning in the cornea leading to visual problems such as myopia and astigmatism. Several treatments, such as lenses and keratoplasty surgeries, were utilized to treat this condition. In this study, we aim to compare deep anterior lamellar keratoplasty (DALK) to penetrating keratoplasty (PK). Methods: We registered our study protocol on PROSPERO (CRD42024559427). Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we searched four electronic databases (PubMed, Scopus, Cochrane Library, and Web of Science) to identify eligible studies reported up to June 2024. Using STATA 17, we reported outcomes as risk ratios (RRs) or mean difference (MD) and confidence intervals (CIs). A P-value ≤.05 is considered as statistically significant. Results: The meta-analysis included a total of 47 studies with a total number of 27,018 eyes. There was no significant difference between PK and DALK in terms of best-corrected visual acuity (BCVA) at 1 year (Hedge's g: 0.03 with 95% CI [−0.13, 0.19], p = .71). PK was associated with a lower risk of graft failure than DALK (log RR: −0.41 with 95% CI [−0.74, −0.08], p = .01). PK was associated with a higher risk of graft rejection than DALK (log RR: 0.85 with 95% CI [0.35, 1.35], p < .001). There was no significant difference between PK and DALK in terms of corneal astigmatism at 1 year (Hedge's g: 0.03 with 95% CI [−0.24, 0.31], p = .82). Conclusion: The visual outcomes such as BCVA, astigmatism, and spherical equivalent refraction, of PK and DALK are comparable. However, PK was associated with a higher risk of complications and rejection episodes, but, a lower risk of graft failure.

Purpose: This study explores the geographic distribution and ethnicity of Iranian patients with Thyroid Eye Disease (TED). It also analyzes semiology, the temporal relationship between thyroid dysfunction and TED, and concomitant diseases. An analysis of ocular visual and motility function in Iranian patients with TED at their initial presentation was also conducted. Methods: Data from the Iran TED Registry (IrTED) was extracted and analyzed to examine the demographics, clinical features, severity, and activity of TED. The study included patients who presented to the Rassoul Akram Hospital oculoplastic clinic in Tehran, Iran, between March 2020 and March 2023. Results: The database (https://orc.iums.ac.ir) included 685 patients (66.56% female), with congenital or acquired thyroid dysfunction/disease diagnosed with a mean age of 34.68 years (SD: 13.95, Range 0-78) and TED onset in the mean age of 37.79 years (SD: 14.06, Range: 2-78). Notably, 10.95% of patients developed TED before thyroid dysfunction, while 62.77% were diagnosed with thyroid dysfunction before the onset of TED. Elevated intraocular pressure (≥21 mmHg) in either eye was observed in 49 (7.15%) of participants, with 32 (4.67%) patients bilaterally involved. The distribution of registered patients by province of birth and ethnicity was also graphed. Semiology, coexisting health conditions, and visual and motility functions of patients were presented in a tabular format. Conclusion: The successful implementation of IrTED contributes to a better understanding of TED epidemiology and semiology within the Iranian population.

Background: Immune checkpoint inhibitors (ICIs) have transformed cancer therapy by targeting key immune pathways such as PD-1, PD-L1, CTLA-4, and LAG-3 to enhance the immune system's ability to combat malignancies. Their use in treating ocular surface tumors is an emerging area of interest, particularly in conjunctival melanoma (CM) and ocular surface squamous neoplasia (OSSN). Some studies have indicated the potential of ICI's in sebaceous gland carcinoma (SeC), conjunctival lymphoma, and Kaposi sarcoma. Purpose: This review aims to evaluate the role of ICIs in treating ocular surface tumors, focusing on their mechanisms of action, clinical outcomes, and therapeutic potential. Methods: A literature review was conducted by searching Pubmed for studies published between January 2014 and October 2024. Studies included were original research, clinical trials, case reports and series, and reviews. Results: ICIs, including pembrolizumab and nivolumab, have shown promising results in CM, achieving tumor regression and disease stabilization in advanced and metastatic cases. ICIs have also demonstrated efficacy in OSSN, particularly in lesions with high tumor mutational burden, with responses ranging from partial to complete resolution. Although clinical data for SeC and conjunctival lymphoma remain limited to isolated reports, these studies suggest a role for ICIs in managing refractory or advanced disease. Conclusion: ICIs hold transformative potential in improving outcomes for ocular surface malignancies, particularly in cases where conventional treatments fail or pose significant morbidity. Despite their promise, challenges persist, including variable response rates, immune-related adverse events, and the need for reliable predictive biomarkers. Comprehensive prospective studies are necessary to refine the application of ICIs, optimize treatment strategies, and expand therapeutic options for these challenging cancers.

Purpose: To assess the efficacy and safety of preoperative intravitreal triamcinolone acetonide (IVTA) combined with internal limiting membrane (ILM) peeling for idiopathic epiretinal membrane (IMEM). Methods: This was a retrospective study. Thirty-six phakic eyes of 35 patients were included in this study. IVTA was administered to 18 patients (18 eyes, Group IVTA) 7 days before vitrectomy, while the other 17 patients (18 eyes, Group no-IVTA) only underwent vitrectomy and ILM peeling. Patients were followed up for at least 6 months. Data on best-corrected visual acuity (BCVA), intraocular pressure (IOP), central macular thickness (CMT), inner retinal thickness (IRT), vascular parameters (measured by optical coherence tomography angiography, OCTA), mean macular sensitivity (MMS), 63% bivariate contour ellipse area (BCEA) and P1 (measured by macular integrity assessment, MAIA) were collected. Results: There were significant differences in BCVA and IRT between the IVTA group and the no-IVTA group at 6 months after surgery (p = .000 and p = .010). The CMT and MMS of the two groups significantly changed from the preoperative values; however, there were no differences between the 2 groups during the entire study period (p = .242 and p = .849). The changes in vascular parameters, including foveal avascular zone (FAZ) area and vessel densities of superficial and deep capillary plexus (SCP VD and DCP VD), in the two groups were not statistically significant. There were no statistically significant differences in 63% BCEA and P1 either. Conclusion: Macular morphology and macular integrity improved after vitrectomy combined with ILM peeling surgery. Compared with the no-IVTA group, preoperative intravitreal triamcinolone acetonide can improve best corrected visual acuity and accelerate the absorption of intraretinal fluid in terms of a significant reduction in IRT.

Juan Murube del Castillo is amongst the foremost prominent figures of Spanish ophthalmology. He was a visionary and an innovator. While several individuals have historically contributed to developing the lacrimal drainage system by innovating surgical instrumentations and techniques,10–15 Murube has carved his unique place in history for posterity. Although several of his works were unique, his single most critical contribution was his vision to form the International Society of Dacryology and Dry Eyes (ISDDE) and then significantly contribute towards its development to a point where it can cater to the needs of dacryologists across the globe.

Purpose: Blepharitis and lower eyelid ectropion are highly prevalent ocular conditions occurring in 37%-46% and 2-3% of the general adult population, respectively. Blepharitis has multifactorial origins and involves anterior and posterior types of eyelid inflammation. Lower eyelid ectropion results in ocular surface exposure, epiphora and chronic conjunctivitis. This study aims to investigate any possible association between both conditions. Methods: Medical records of 37,692 consecutive patients examined at a single medical screening center between 2001-2020 were retrospectively analyzed. Main outcome measures: The prevalence of lower eyelid ectropion and of blepharitis, a possible association between the two, and the relation of each to age and sex. Results: A total of 35,670 patients were included. Ectropion was diagnosed in 69 patients (0.2%), and blepharitis in 4725 patients (13.2%). Male sex was more prevalent for each pathology (88.4% ectropion, and 85% blepharitis, p < .001). Older age was associated with each diagnosis (77.3 years for ectropion patients vs. 52.2 years for the general screened population and 60.5 years for blepharitis patients vs 52.2 years for the general screened population p < .001). The prevalence of ectropion was significantly higher in patients with coexisting blepharitis compared to those without (0.8% vs. 0.1%, respectively, p < .001). Conclusions: Ectropion was significantly more prevalent in patients with blepharitis. Both conditions were associated with older age and male sex. This coexistence can aid in decision making of early surgical intervention of lower eyelid ectropion as well as the approach to medical treatment of blepharitis.

A review of Rose’s contributions reveals that he is an astute clinician and a visionary Dacryologist. While several individuals have historically contributed to the understanding of lacrimal physiology and management of functional epiphora,Citation21–25 Rose provided evidence-based insights and added fresh perspectives and new ways to approach complex lacrimal disorders. Several of Rose’s observations, including those on lacrimal physiology and tear flow dynamics, continue to hold high value several decades after their initial description. His contributions have carved a special place for themselves, and his name is etched in the history of lacrimal disorders. Rose is still active, and the lacrimal world immensely benefits from his wisdom. We can, for sure, expect several more benchmark works from his clinics.

Purpose: Standardized data collection is needed to improve research for rare diseases. In this manuscript, we describe our experience establishing the Prospective Ocular Tumor Study (POTS). Methods: The ongoing POTS captures all patients with an ocular tumor seen on the Ocular Oncology Service at Mayo Clinic Rochester and collects patient demographics, tumor features, treatment, and outcomes. This manuscript reports data collected from July 2019-July 2024. Results: During a 5-year time period, 1,766 patients enrolled in the database, with 975 (55%) females, 1,732 (98%) white race, and mean age 61.5 years. The most frequent tumor types were choroidal melanoma (n = 610 [34%]), choroidal nevus (n = 575 [32%]), iris nevus (n = 95 [5.3%]), iris melanoma (n = 46 [2.6%]), and vitreoretinal lymphoma (n = 46 [2.6%]). Conclusion: The POTS is a valuable source of detailed, longitudinal data on rare ocular tumors. Expanding standardized data collection across multiple centers will facilitate improved outcomes research in ocular oncology.

Objective: Ciliary body medulloepithelioma (CBME), a pediatric intraocular tumor with potential for locally aggressive behavior and metastasis, may present with a diverse spectrum of clinical and histopathologic features leading to diagnostic and management challenges. Examination of unusual CBME cases highlights challenges and modern diagnostic techniques which facilitate accurate diagnosis and guide management. Methods: A retrospective clinicopathologic analysis of 6 patients with unusual clinical or pathologic features of CBME was performed. Results: The mean duration of delay in accurate diagnosis was 5.7 years (SD: 8.2, median: 3, range: 0-22). All patients developed cataract, 4 (67%) were diagnosed with glaucoma, and 4 (67%) underwent surgery prior to accurate diagnosis. At initial presentation, only one patient with a known history of genetically confirmed DICER1 syndrome underwent appropriate imaging leading to a timely identification of a ciliary body mass and no delay in diagnosis. Following identification of intraocular mass, 4 (67%) patients underwent enucleation. Two patients (33%) underwent exenteration for extraocular extension of CBME. Initial histopathologic differential diagnosis included CBME, melanoma, adenoma or adenocarcinoma of the pigmented ciliary body epithelium, retinoblastoma, sarcoma, and malignant teratoma. Immunohistochemistry and genetic testing assisted in the diagnosis of CBME. Two patients (33%) had a germline DICER1 variant; this was known prior to CBME diagnosis in one patient and discovered after CBME diagnosis in the second patient. Conclusion: This series highlights the unusual clinical and histopathologic features of CBME that contribute to delays in diagnosis. Modern aids including genetic testing, ancillary imaging studies, and immunohistochemistry facilitate a timely accurate diagnosis of CBME and guide management.

A review of Paulsen’s contributions reveals that he is an ingenious anatomist and a brilliant basic scientist. While several individuals have historically contributed to understanding lacrimal drainage anatomy and pathophysiology, Paulsen’s contributions stand tall and have acted as a catalyst to revolutionize the way that we appreciate tear ducts today. Through his enormous body of work, he also successfully drew attention to the interactions between the lacrimal drainage and the ocular surface. His collaborative efforts have helped to accelerate the development of lacrimal sciences rapidly. Through his contributions, Paulsen has etched his name in the history of lacrimal drainage disorders. I can say with full responsibility that no historical documentation of lacrimal drainage sciences can be complete without Friedrich Paul Paulsen. Fortunately, he is still young and active, and the lacrimal world expects him to contribute further to the fundamental understanding of lacrimal sciences.