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Kaustubh Mulay,1 Mohammad Javed Ali,2 Vijay Anand Reddy,2 Santosh G Honavar21Ocular Pathology Service, LV Prasad Eye Institute, Hyderabad, Andhra Pradesh, India; 2Oncology Service, LV Prasad Eye Institute, Hyderabad, Andhra Padresh, IndiaAbstract: Ocular involvement by plasmablastic lymphoma is extremely rare with very few reports in the literature. Its morphological and immunological resemblance to plasma cell myeloma makes it a diagnostic challenge, while its clinical course, which is characterized by recurrence and death, makes therapy a challenge for clinicians. We present three cases of plasmablastic lymphoma, each of which has distinct clinicoradiological features, and we also review the literature on orbital plasmablastic lymphomas.Keywords: plasmablastic lymphoma, ocular, myeloma, orbit

We report a rare presentation of an initially misdiagnosed case of a pseudotumor, which on histopathology was diagnosed as bilateral breast metastases of lobular carcinoma involving multiple extraocular muscles. A 61-year-old lady presented with external ophthalmoplegia and diplopia. Incisional biopsy was performed using a lid crease approach and the patient received radiotherapy and hormonal therapy. Following prolonged hormonal therapy, complete remission was achieved, with improvement in ocular motility and resolution of diplopia, about 18 months after the initial presentation. Multiple extraocular muscle involvement by breast carcinoma metastasis is very rare and should be considered in the differential diagnosis, especially in patients with a prior history of breast carcinoma. [ABSTRACT FROM AUTHOR]

Purpose: To describe the surgical technique using a 10/14 French silicone urinary catheter as a novel tissue expander for repair of defects after removal of eyelid tumors. This device recruits additional tissue by tissue expansion for repair of large eyelid defects., Methods: A prospective noncomparative, interventional case study was conducted over a period of 3 years in which 30 patients were enrolled. All the 30 patients were selected for upper or lower lid Tenzel rotational flap after removal of large malignant tumor, using the tissue expander., Results: A 10/14-French silicone urinary catheter was placed in the periocular region and expanded by graded inflation with normal saline to a total volume of 10 or 14 ml, respectively, over a period of 3 days. On the fourth day before the planned surgery, the catheter balloon was deflated and a modified Tenzel rotational flap was performed to close the defect. Postoperative blink reflex was preserved. There were no cases of wound dehiscence. There was a significant reduction of incision length in upper and lower lid reconstruction with modified Tenzel flap reconstruction., Conclusion: This approach allows for temporary tissue expansion, permitting the closure of large eyelid defects, typically not possible with a conventional Tenzel flap. This also provides excellent functional outcomes without closing patients' eyelids (which limits vision for weeks to months) as is typical of other reconstructive modalities (e.g. Cutler Beard procedure). The device is cost-effective and readily available. The authors believe it is an excellent alternative in the developing world where access to more expensive options is limited., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Purpose: With the increased survival of retinoblastoma (RB) patients, it is important to evaluate the quality of life (QoL) of RB survivors as well as caregivers to provide comprehensive care to the children and caregivers. This study aims to assess the QoL of survivors of RB, as perceived by parents and the self-report by patients, through a pediatric QoL (PEDs-QoL) questionnaire., Methods: The study cohort included 86 RB survivors, 86 age-matched controls, and their primary carers. PedsQL 4.0 generic core scale and structured interview were administered. QoL in physical, social, emotional, and school health was evaluated and correlated with clinical and sociodemographic parameters., Results: The mean age of the RB survivors was 5.7 years with an M:F ratio of 1.1:1. Disease was bilateral in 79% of cases. About 45% (39/86) underwent enucleation, while others received combination therapy (16; 18%), chemotherapy (30; 34%), and radiation (1; 1%). As reported by parents, the QoL of physical health domain of RB survivors was 70.2 SD ± 27.8 and 96.15 ± 13 SD, emotional health was 72.1 ± 27.4 SD and 94.4 ± 12.5 SD, social health was 80.4 ± 24.9 SD and 98.6 ± 6.2 SD; and school health was 71.9 ± 2 6.5 SD and 96.1 ± 12.2 SD. As per the self-report perception, the QoL of physical health was 68.2 ± 27.8 SD and 96.2 ± 13 SD, emotional health was 66.2 ± 28.4 SD and 95.3 ± 12.5 SD, social health was 69.5 ± 24.9 SD and 98.7 ± 6.2 SD, and school health was 63.5 ± 26.5 SD and 95.1 ± 12.2 SD. There was a significant relationship between enucleation and QoL domains, where χ2 = 67.75, degrees of freedom (df) = 36, and P < 0.01. There was a significant association between vision in the better eye (6/18 or better = 8, 6/18-6/60 = 8, 3/60 or worse = 42) and QoL scores (χ2 = 95.36, df = 62, P < 0.01). There was a substantial association between socioeconomic status and QoL domains, where χ2= 88.5, df = 56, P < 0.01., Conclusion: The results of the study showed that the QoL of parents of RB survivors and self-proxy reports were negatively affected in many ways, including physical, social, emotional, and school-related dimensions. Despite the small differences, self-proxy reports indicated a lower QoL than the parents' group. The study findings indicate that there are notable correlations between enucleation and visual acuity less than 6/18, as well as socioeconomic status, with various aspects of QoL domains among individuals who have survived RB., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Purpose: To study the long-term efficacy of intravitreal topotecan (IVT) for vitreous seeds in eyes with retinoblastoma and risk factors for their recurrence., Design: Retrospective, non-comparative, interventional study., Participants: Ninety-one eyes of 90 patients with retinoblastoma treated between January 2013 and April 2019., Methods: Patients with recurrent or refractory vitreous seeds after completion of intravenous or intra-arterial chemotherapy were treated with IVT (30 μg/0.15 ml) by the safety-enhanced technique. The injection was repeated every 4 weeks until the regression of seeds. Patients with a minimum follow-up of 12 months were included in the analysis., Main Outcome Measures: Primary outcome measures were vitreous seed regression and eye salvage. Secondary outcomes were risk factors for vitreous seed recurrence after treatment with IVT, vision salvage, and complications of IVT., Results: The median age of the patients was 18 months, with most having group D (n = 58 [64%]) and group E (n = 26 [29%]) retinoblastoma. Vitreous seeds were refractory in 46 eyes (51%) and recurrent in 45 eyes (49%). A total of 317 IVT injections were administered, with the median being 3 injections. The median number of IVT injections required was 2.5 injections for dust, 3 injections for sphere, and 5 injections for cloud morphologic features. Recurrence of vitreous seeds after IVT was seen in 17 eyes (19%) at a mean follow-up of 7.9 months. At a mean follow-up 34 months, vitreous seed regression was achieved in 88 eyes (97%) and eye salvage was achieved in 77 eyes (85%). Older age (P = 0.018) and recurrence of retinal tumor (15/17 eyes; P < 0.01) significantly increased the risk of vitreous seed recurrence. Cataract was the most common complication seen in 17 eyes (9%)., Conclusions: Intravitreal topotecan at an every 3- to 4-week regimen is effective against both refractory and recurrent vitreous seeds. The vitreous seed morphologic features correspond to the number of injections required for regression. Increasing age and recurrence of retinal tumor increase the risk of vitreous seed recurrence after treatment with IVT., Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

There have been rapid advancements in the field of ocular oncology for the diagnosis and management of intraocular, adnexal, and orbital tumors. Targeted therapy is in the forefront of medical research in all fields including ocular oncology. Targeted therapy include drugs that target specific genetic mutations, pathways or proteins involved in the development of cancer. In contrast to traditionally used chemotherapy, drugs used in targeted therapy are highly specific for tumor cells and preserve the function of normal cells. This review aims to familiarize ophthalmologists with the drugs that are currently approved or undergoing clinical trials for use in ocular oncology. Targeted therapy is particularly useful for locally advanced or metastatic tumors, including but not limited to eyelid and periocular basal cell carcinoma, periocular cutaneous and conjunctival squamous cell carcinoma, ocular adnexal lymphoma, conjunctival melanoma, and uveal melanoma. The results are promising with improved survival outcomes and better tolerability than chemotherapeutic drugs., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

The coronavirus disease 2019 (COVID-19) pandemic caused by the severe acute respiratory syndrome coronavirus 2 was one of the most devastating public health issues in recent decades. The ophthalmology community is as concerned about the COVID-19 pandemic as the global public health community is, as COVID-19 was recognized to affect multiple organs in the human body, including the eyes, early in the course of the outbreak. Ophthalmic manifestations of COVID-19 are highly variable and could range from mild ocular surface abnormalities to potentially sight and life-threatening orbital and neuro-ophthalmic diseases. Furthermore, ophthalmic manifestations may also be the presenting or the only findings in COVID-19 infections. Meanwhile, global vaccination campaigns to attain herd immunity in different populations are the major strategy to mitigate the pandemic. As novel vaccinations against COVID-19 emerged, so were reports on adverse ophthalmic reactions potentially related to such. As the world enters a post-pandemic state where COVID-19 continues to exist and evolve as an endemic globally, the ophthalmology community ought to be aware of and keep abreast of the latest knowledge of ophthalmic associations with COVID-19 and its vaccinations. This review is a summary of the latest literature on the ophthalmic manifestations of COVID-19 and the adverse ophthalmic reactions related to its vaccinations., (Copyright © 2023 Asia-Pacific Academy of Ophthalmology. Published by Wolters Kluwer Health, Inc. on behalf of the Asia-Pacific Academy of Ophthalmology.)

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Aims: To examine whether the specific location of ocular adnexal lymphoma (OAL) and the American Joint Committee on Cancer (AJCC) TNM tumour stage are prognostic factors for mortality in the main OAL subtypes., Methods: Clinical and survival data were retrospectively collected from seven international eye cancer centres. All patients from 1980 to 2017 with histologically verified primary or secondary OAL were included. Cox regression was used to compare the ocular adnexal tumour locations on all-cause mortality and disease-specific mortality., Results: OAL was identified in 1168 patients. The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n=688, 59%); follicular lymphoma (FL) (n=150, 13%); diffuse large B-cell lymphoma (DLBCL) (n=131, 11%); and mantle cell lymphoma (MCL) (n=89, 8%). AJCC/TNM tumour-stage (T-stage) was significantly associated with disease-specific mortality in primary ocular adnexal EMZL and increased through T-categories from T1 to T3 disease. No associations between AJCC/TNM T-stage and mortality were found in primary ocular adnexal FL, DLBCL, or MCL. EMZL located in the eyelid had a significantly increased disease-specific mortality compared with orbital and conjunctival EMZL, in both primary EMZL and the full EMZL cohort. In DLBCL, eyelid location had a significantly higher disease-specific mortality compared with an orbital or lacrimal gland location., Conclusion: Disease-specific mortality is associated with AJCC/TNM T-stage in primary ocular adnexal EMZL patients. Lymphoma of the eyelid has the highest disease-specific mortality in primary EMZL, and in the full cohort of EMZL and DLBCL patients., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

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Retinoblastoma is a retinal cancer that affects children and is the most prevalent intraocular tumor worldwide. Despite tremendous breakthroughs in our understanding of the fundamental mechanisms that regulate progression of retinoblastoma, the development of targeted therapeutics for retinoblastoma has lagged. Our review highlights the current developments in the genetic, epigenetic, transcriptomic, and proteomic landscapes of retinoblastoma. We also discuss their clinical relevance and potential implications for future therapeutic development, with the aim to create a frontline multimodal therapy for retinoblastoma., Competing Interests: None

Background: Pigmented lesions in the conjunctiva can be baffling to both the patients and the treating ophthalmologist because of their varied range of presentation and overlapping clinical features. The lesions range from incidental pigment deposition such as mascara and complexion-associated melanosis to malignant melanoma which poses a risk to life. Similarly, the management ranges from observation at regular intervals to aggressive surgery like exenteration., Purpose: We wanted to present a crisp and precise video of the good, bad, and ugly pigmented lesions of the conjunctiva, highlighting their specific clinical features important for the diagnosis and their management., Synopsis: This video describes the myriad of pigmented conjunctival lesions, their diagnostic characteristics, and management based on oncological principles., Link: https://drive.google.com/file/d/1BYJ51rQtqjwM6e73BwrrLqdC1EoXA8Eu/view?usp=sharing., Highlights: : Pigmented lesions can have variable presentation and close mimics, therefore, it is important to differentiate and identify the lesions accurately. This video highlights different pigmented lesions and their individual characteristic features. Video link https://youtu.be/m9tt7dx9SWc., Competing Interests: None

Background: : In 1978, the advent of magnetic resonance imaging (MRI) was a remarkable volte-face in the world of diagnostics. Employing the phenomenon of nuclear resonance enables us to exploit the properties of differential protons in living tissues. The ability of providing higher and variable contrast and the absence of ionizing radiations make it superior to computed tomography. Being the diagnostic tool of choice, it is an indispensable part of assessment of the location and characteristics of different ocular and orbital pathologies (vascular, inflammatory, and neoplastic)., Purpose: : The intrinsic and extrinsic properties of MRI provide multi-parametric imaging, making it of paramount importance in ophthalmological evaluation. Also, MRI-dynamic color mapping provides non-invasive and quantitative assessment of soft tissues in motion. An in-depth knowledge of the basic principle and technique of MRI aids in diagnosing as well optimal planning of surgical interventions., Synopsis: : In this video, we shall be displaying the anatomical, clinical, and radiological aspects of MRI with an overlap to make it easier to understand the implications of this miraculous invention., Highlights: : A good understanding of MRI analysis makes the ophthalmologists independent and helps in ruling out the differential diagnoses, exact extent and invasion, precise surgical planning and therefore, avoiding tragic outcomes. This video is an attempt to simplify and emphasize on the importance of MRI interpretation for an ophthalmologist. Video link https://youtu.be/r5dNo4kaH8o., Competing Interests: None

Background: Plaque brachytherapy is an evolving, yet effective globe and vision-sparing modality for the treatment of intraocular tumors by transscleral irradiation of the tumor base with a radioactive implant. The American Brachytherapy Society (ABS) along with the collaboration of the international multicenter Ophthalmic Oncology Task Force (OOTF) was assembled to reach a consensus regarding establishing practice guidelines and setting standards of care for intraocular tumors. The advent of plaque brachytherapy has revolutionized the outcomes of intraocular tumors, thus ensuring globe salvage, reducing morbidity and mortality, and avoiding cosmetic disfigurement. A well-planned dosimetry for plaque brachytherapy results in achieving local tumor control and excellent prognosis., Purpose: This technique provides the advantage of focal radiation, thus eliminating the damage to the adjacent structures, minimal periorbital tissue damage, absence of cosmetic disfigurement owing to lack of retarded bone growth as seen in external beam radiotherapy. Thus, it reduces the risk of metastasis, and with the recent advances, it provides a shorter duration of treatment., Synopsis: : In this video, we shall display the concept of plaque brachytherapy, the various types of plaques available, different radiations sources, planning dosimetry and calculations, target disease spectrum, surgical placement, and post-radiation outcomes in terms of local tumor control and prognosis., Highlights: This video highlights the history, basic principles and techniques of plaque brachytherapy and provides an understanding of its applications in the world of ocular oncology., Video Link: https://youtu.be/7PX0mDQETRY., Competing Interests: None

Background: : A good anesthesia not only makes the patient comfortable during surgery, but also has a huge impact on the postoperative recovery. It also makes the operating surgeon carry out each step of the surgery precisely and beautifully. The art of giving a good local anesthesia is to be learnt and practiced not only by anesthetists, but also by the practicing ophthalmologists., Purpose: : This video gives an overview of anatomy in terms of the nerve supply of the orbit, the surface marking, and the techniques of giving regional and nerve blocks., Synopsis: : In this video, we describe the anatomy, the surface marking, the technique of regional anesthesia including peribulbar, retrobulbar, and subtenon blocks and of nerve blocks, specifically of facial, frontal nerve and its branches, infraorbital, nasociliary, infratrochlear, and dorsal nasal nerves, with their application in ocular plastic surgery., Highlights: : This video highlights the essence of providing appropriate and good anesthesia so that the surgeon works in an optimal field with maximum comfort to the patients. Video link https://youtu.be/h8EgTMQAsyE., Competing Interests: None

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Background/aim: To evaluate the safety and efficacy of ruthenium-106 (Ru-106) plaque brachytherapy in managing invasive ocular surface squamous neoplasia (OSSN)., Methods: This is a retrospective, non-comparative, interventional case series of 42 eyes with OSSN with histopathologically-proven corneal stromal and/or scleral invasion that underwent Ru-106 plaque brachytherapy. Main outcome measures were tumour regression, eye salvage, final visual acuity, treatment complications and metastasis., Results: At presentation, the mean tumour basal diameter was 9.3 mm (range 5-26 mm) and thickness 3.1 mm (range 1.5-11 mm). Prior treatment included excision biopsy in two patients (5%), incision biopsy and topical interferon in one each (2%). Following excision with 4 mm clinically clear margins, corneal stromal and/or scleral invasion of OSSN was confirmed in all 42 cases, with the excised base showing invasive squamous cell carcinoma. A total dose of 5000 cGy over a mean duration of 19.7 hours (range 7-41 hours) was provided to an axial depth of 2 mm using Ru-106 surface plaque. Over a mean follow-up of 36.9 months (range 22.3-72 months), complete tumour regression was achieved in all eyes (100%). Two eyes (5%) showed conjunctival tumour growth remote from the site of prior treatment. Visual acuity was maintained at ≥20/200 in 35 eyes (83%), with a loss of >2 Snellen lines in 1 eye (2%). There was no evidence of regional lymph node or systemic metastasis., Conclusion: Histopathology-guided use of Ru-106 surface plaque brachytherapy is a safe and an effective adjuvant therapy in the management of corneal stromal and/or scleral invasion of OSSN., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

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The rise of primary topical monotherapy with chemotherapeutic drugs and immunomodulatory agents represents an increasing recognition of the medical management of ocular surface squamous neoplasia (OSSN), which may replace surgery as the standard of care in the future. Currently, there is no consensus regarding the best way to manage OSSN with no existing guidelines to date. This paper seeks to evaluate evidence surrounding available treatment modalities and proposes an approach to management. The approach will guide ophthalmologists in selecting the most appropriate treatment regime based on patient and disease factors to minimize treatment related morbidity and improve OSSN control. Further work can be done to validate this algorithm and to develop formal guidelines to direct the management of OSSN.

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