Ayushi Agarwal, Vijitha S Vempuluru, Faraz Ali Mohammad, Mohammed Hasnat Ali, Vijay Anand Reddy Palkonda, Swathi Kaliki
{"title":"Primary orbital and adnexal rhabdomyosarcoma: a study of 54 Asian Indian patients.","authors":"Ayushi Agarwal, Vijitha S Vempuluru, Faraz Ali Mohammad, Mohammed Hasnat Ali, Vijay Anand Reddy Palkonda, Swathi Kaliki","doi":"10.1080/01676830.2024.2419058","DOIUrl":"10.1080/01676830.2024.2419058","url":null,"abstract":"<p><strong>Purpose: </strong>To describe the clinical presentation, management, and outcomes of orbital and adnexal rhabdomyosarcoma (RMS) in an Asian-Indian cohort and analyze the factors predictive of outcomes.</p><p><strong>Methods: </strong>Retrospective interventional case series of 54 patients of histopathology-proven RMS.</p><p><strong>Results: </strong>The mean age of presentation with RMS was 10 years (median, 7 years; range, <1-54 years). The most common tumor location was the superonasal quadrant (<i>n</i> = 21, 39%). Extensions beyond the orbit into the sinuses, temporal fossa, and brain were seen in 15 (28%), 3 (6%), and 8 (15%), respectively. First-line treatment modalities included a combination of surgical debulking, chemotherapy, and external beam radiotherapy in 20 (37%) patients; chemotherapy and radiation in 20 (37%); complete surgical excision and chemotherapy in 3 (6%); orbital exenteration, chemotherapy, and external beam radiotherapy in 1 (2%); chemotherapy alone in 1 (2%) patient, and 9 (17%) patients were lost to follow up. By Intergroup Rhabdomyosarcoma Study Group (IRSG) grouping, tumors belonged to groups I (<i>n</i> = 2,4%), II (<i>n</i> = 13, 29%), and III (<i>n</i> = 30, 67%). Kaplan-Meier survival estimates for tumor recurrence, lymph node metastasis, systemic metastasis, and death (<i>n</i> = 45) were 84%, 82%, 95%, and 95% at 1 year, 69%, 76%, 81%, and 77% at 5 years, 69%, 76%, 81%, and 77% at 10 years, respectively. On multivariate regression, age >18 years was found to have a significantly increased risk of locoregional LN metastasis (<i>p</i> = 0.005).</p><p><strong>Conclusion: </strong>Despite aggressive treatment modalities, the 5-year disease-specific survival rate was 77% in Asian-Indian cohort. Age > 18 years at presentation was associated with a poorer prognosis.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"257-266"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142548283","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Reza Jafari, Ali Shushtari, Hanie Ahmadi, Ahmad Ahmadzade Amiri, Amir Veisi
{"title":"Solitary neurofibroma confined to inferior rectus muscle tendon: a case report.","authors":"Reza Jafari, Ali Shushtari, Hanie Ahmadi, Ahmad Ahmadzade Amiri, Amir Veisi","doi":"10.1080/01676830.2024.2377248","DOIUrl":"10.1080/01676830.2024.2377248","url":null,"abstract":"<p><p>The present study reports a case of solitary neurofibroma attached to the Inferior Rectus (IR) muscle tendon in a 24-year-old healthy woman and reviews the relevant literature regarding the clinical presentation, diagnosis, and management of this uncommon tumor. The patient underwent successful surgical resection of the tumor, leading to the resolution of associated symptoms (left lower eyelid protrusion and redness). Pathological examination confirmed the diagnosis of neurofibroma based on characteristic histopathological and immunohistochemical markers. This case report underscores the rarity of solitary neurofibromas and primary neoplasms of orbit and ocular adnexa. We also discuss the background of solitary neurofibromas originating from orbit and ocular adnexa. The successful management of this case through surgical resection highlights the importance of accurate diagnosis and tailored treatment strategies. To the best of our knowledge, this is the first reported solitary neurofibroma confined solely to the IR tendon.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"317-320"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141634988","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hays T Cape, Lalita Gupta, Cody C Blanchard, Flavius D Raslau, Peter J Timoney
{"title":"Tumor versus bland thrombus: diffuse large B-cell lymphoma presenting as cavernous sinus and left superior ophthalmic vein thrombosis.","authors":"Hays T Cape, Lalita Gupta, Cody C Blanchard, Flavius D Raslau, Peter J Timoney","doi":"10.1080/01676830.2024.2393416","DOIUrl":"10.1080/01676830.2024.2393416","url":null,"abstract":"<p><p>A 66-year-old female with a history of sinusitis presented with persistent diplopia and worsening eyelid swelling. Examination revealed bilateral vision loss, cranial nerve palsies, left-sided chemosis, proptosis, and edema. Initial imaging showed sphenoid sinus opacification, bilateral ethmoid disease, lack of filling of bilateral cavernous sinuses, and partial thrombosis of the left superior ophthalmic vein. The sinus findings were evaluated with endoscopic sinus surgery, which was unremarkable. Subsequent orbital imaging suggested the lesions were in keeping with tumor thrombus as opposed to bland thrombus. CT scans revealed a large mass in the right axilla, which was biopsied and confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL). The patient was treated with anticoagulation, steroids, and chemotherapy resulting in marked improvement. Our report highlights a rare case of tumor thrombus from DLBCL causing bilateral cavernous sinus thrombosis and, to the best of our knowledge, the first documented case of superior ophthalmic vein thrombosis from tumor thrombus.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"348-352"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142082182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hesham A Ibrahim, Eman M Salem, Ibrahim Y Allam, Heba N Sabry
{"title":"Comparison of eyelid function following frontalis suspension and levator dissection-resection in congenital ptosis with poor levator function.","authors":"Hesham A Ibrahim, Eman M Salem, Ibrahim Y Allam, Heba N Sabry","doi":"10.1080/01676830.2024.2399665","DOIUrl":"10.1080/01676830.2024.2399665","url":null,"abstract":"<p><strong>Purpose: </strong>A levator dissection-resection technique (LDR) in which the levator is dissected free from all fibrous attachments including Whitnall's ligament was compared to two commonly used frontalis-based procedures in the management of congenital ptosis with poor levator function.</p><p><strong>Methods: </strong>Thirty patients having congenital ptosis with poor levator function were randomized to one of the three surgical groups (ten patients for each group), namely, frontalis sling (FS), frontalis advancement flap (FAF), and (LDR) technique. Marginal reflex distance-one (MRD-1), levator function, symmetry in different levels of vertical gaze, lagophthalmos, and any other surgical complications were assessed 6 months following the intervention.</p><p><strong>Results: </strong>Patients in the three groups achieved statistically significant improvement in MRD-1 in the primary position of gaze (<i>p</i> = 0.001 for FS, 0.003 for FAF, 0.001 for LDR). Patients who underwent a frontalis-based procedure acquired an additional ability to elevate the upper eyelid by using their eyebrows. Patients who underwent LDR technique have acquired an additional mean of 5.79 ± 1 mm improvement in levator function with better symmetry during up and down gaze in unilateral cases. Patients from all groups had an equal degree of lagophthalmos with forced eyelid closure, and during sleep.</p><p><strong>Conclusion: </strong>Patients with ptosis and poor levator function who were managed with LDR technique achieved a similar degree of eyelid elevation in the primary gaze to that of frontalis-based procedures, acquired additional levator function, achieved more symmetry in up and down gaze in unilateral cases, and had no additional risk to the cornea.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"299-305"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142298554","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Chia W Hsu, Cornelia W Peterson, Charles G Eberhart, Christian F Meyer, Deborah K Armstrong, Katie Fiallos, Ashley A Campbell
{"title":"Ocular adnexal sebaceous carcinoma in a patient with Li-Fraumeni syndrome.","authors":"Chia W Hsu, Cornelia W Peterson, Charles G Eberhart, Christian F Meyer, Deborah K Armstrong, Katie Fiallos, Ashley A Campbell","doi":"10.1080/01676830.2024.2382268","DOIUrl":"10.1080/01676830.2024.2382268","url":null,"abstract":"<p><p>Li-Fraumeni syndrome (LFS) is caused by a pathogenic germline variant at the <i>TP53</i> locus and is associated with an increased predisposition to a variety of cancers. The neoplasms most frequently associated with LFS are sarcomas, breast cancer, brain tumors, and adrenocortical carcinomas. In this case report, we present a 43-year-old male diagnosed with an ocular adnexal sebaceous carcinoma of the right upper eyelid who was confirmed to have LFS with subsequent genetic testing. The mutational profile of both the patient's genetic screen and tumor sequencing were congruent, demonstrating the same pathogenic loss-of-function <i>TP53</i> variant. This case report highlights the importance of pursuing genetic testing in patients with a history of multiple tumor types, particularly those with uncommon diagnoses. In this case, confirmation of LFS had important implications for personalized patient care, including identification of contraindicated treatment interventions and the imaging modalities necessary for vigilant follow-up screening.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"321-325"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141789414","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Josefina Herrera, Gerard Reid, Peter McCluskey, Krishna Tumuluri
{"title":"Orbital necrobiotic xanthogranuloma with necrotizing scleritis and absent cutaneous manifestations.","authors":"Josefina Herrera, Gerard Reid, Peter McCluskey, Krishna Tumuluri","doi":"10.1080/01676830.2024.2387105","DOIUrl":"10.1080/01676830.2024.2387105","url":null,"abstract":"<p><p>Necrobiotic xanthogranuloma (NXG) is a rare systemic disease, that commonly manifests with orbital and ocular adnexal involvement, presenting with periocular yellow papules and plaques that may ulcerate. Periorbital skin lesions are a hallmark of the disease, and in their absence the diagnosis may be delayed, preventing prompt systemic evaluation and appropriate treatment of this condition. We report a unique case of a 58-year-old female patient with NXG that presented with severe bilateral necrotizing scleritis, left orbital mass and no cutaneous manifestations of NXG. This case highlights the importance of considering NXG, despite absence of skin lesions, when other ophthalmological manifestations are present. Recognition of NXG is crucial due to the high lifelong risk of developing hematological malignancy.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"343-347"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141898647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Óscar Felguera-García, Marina Dotti-Boada, Sara Labay-Tejado, Eduard Millán-Valbuena, Silvia Feu-Basilio, Sara Marín-Martínez, Marta Pazos, Jessica Matas Fassi
{"title":"Canalicular obstructions after monkeypox infection.","authors":"Óscar Felguera-García, Marina Dotti-Boada, Sara Labay-Tejado, Eduard Millán-Valbuena, Silvia Feu-Basilio, Sara Marín-Martínez, Marta Pazos, Jessica Matas Fassi","doi":"10.1080/01676830.2024.2384049","DOIUrl":"10.1080/01676830.2024.2384049","url":null,"abstract":"<p><p>We report two cases of lacrimal obstruction after ocular infection with monkeypox virus (MPX) in the 2022 outbreak. One of them was a distal canalicular obstruction and the other was a proximal canalicular obstruction. In the first days of MPX they presented with conjunctivitis and periocular skin vesicles. Several months after the ophthalmic condition was cured, they showed persistence of epiphora, and the lacrimal problem was diagnosed. The photographs taken during the inflammation of the anterior pole were reviewed and vesicles located in the same area as the canalicular damage were observed. One patient underwent a canaliculodacryorhinostomy and the other an exploratory punctoplasty. Both surgical operations failed to restore normal tear flow. Lacrimal drainage disorders related to ocular MPX have not yet been described. And, in addition, these cases are the first relation of viral vesicular skin lesions and canalicular obstructions.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"332-334"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141861293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Congenital orbital respiratory epithelial cyst with anomalous maxillary antrum in the absence of bony defect.","authors":"Anthony Yao, Sepideh Amin, Raman Malhotra","doi":"10.1080/01676830.2024.2373811","DOIUrl":"10.1080/01676830.2024.2373811","url":null,"abstract":"<p><p>A 23-year-old female symptomatic with unilateral proptosis with superior globe displacement and hypertropia was found to have a multiloculated cystic lesion with bony lining arising from the inferomedial orbit. The adjacent maxillary sinus was markedly smaller on the affected side, with no defect of the wall on radiographic or intraoperative examination. Surgical excision and histological analysis of the lesion demonstrated an orbital respiratory epithelial cyst. A literature review of congenital orbital respiratory epithelial cysts is presented. To our knowledge, this is the first published case of choristomatous orbital respiratory epithelial cyst associated with ossification, and a primary or secondary anomaly of the adjacent sinus without bony defect.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"314-316"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141493861","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Niloufar Bineshfar, Leo Meller, Benjamin I Meyer, Wendy W Lee
{"title":"Impact of phenylephrine-induced Hering's response on Müller's muscle-conjunctival resection outcomes.","authors":"Niloufar Bineshfar, Leo Meller, Benjamin I Meyer, Wendy W Lee","doi":"10.1080/01676830.2024.2435928","DOIUrl":"10.1080/01676830.2024.2435928","url":null,"abstract":"<p><strong>Purpose: </strong>To evaluate the impact of preoperative phenylephrine (PE)-induced Hering's response on postoperative outcomes in patients undergoing unilateral Müller's muscle-conjunctival resection (MMCR) for blepharoptosis correction.</p><p><strong>Methods: </strong>Sixty-five consecutive patients (65 eyes) undergoing unilateral MMCR between January 2016 and December 2023 were included in this retrospective single-surgeon study. The main outcome measure was the margin-reflex distance 1 (MRD1) at postoperative month 1 visit. Logistic regression analysis was conducted to identify factors influencing postoperative Hering's response.</p><p><strong>Results: </strong>A preoperative post-PE Hering's response was observed in 40.0% of patients. At 1 week and 1 month postoperatively, Hering's response was recorded in 20.0% and 21.5% of cases, respectively. Regression analysis revealed a significant correlation between preoperative post-PE Hering's and post-MMCR Hering's response at postoperative month 1 visit (crude odds ratio (OR): 8.80, <i>p</i> = .003; adjusted OR: 30.43, <i>p</i> = .002).</p><p><strong>Conclusion: </strong>Preoperative post-PE Hering's response is a significant predictor of postoperative Hering's response in unilateral MMCR.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"289-294"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142830425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Matthew Fenech, Ayodeji Ajanaku, James Hsuan, Austin McCormick, Simon Shamas, Nima Ghadiri
{"title":"Immune checkpoint inhibitors and the orbit; two cases of reactive dacryoadenitis.","authors":"Matthew Fenech, Ayodeji Ajanaku, James Hsuan, Austin McCormick, Simon Shamas, Nima Ghadiri","doi":"10.1080/01676830.2024.2385010","DOIUrl":"10.1080/01676830.2024.2385010","url":null,"abstract":"<p><p>Immune checkpoints refer to mechanisms entrusted with the modulation of immune responses in peripheral tissues and are required for minimising collateral damage. Immune checkpoint inhibitors (ICPi) work through numerous pathways, including the anti-CTLA-4 (cytotoxic T-lymphocyte-associated protein 4), anti-PD-1 (programmed cell death protein 1) and the PD-L1 (protein cell death protein-ligand-1) pathways. They are proving to be an exciting therapeutic avenue in the attempt to activate anti-tumour activity. Ipilimumab is a fully human monoclonal antibody working on the anti-CTLA-4 pathway, while nivolumab and pembrolizumab are humanised monoclonal IgG4 antibodies that work on the PD-1 pathway. Despite a growing body of research pertinent to these novel therapies, early indications show that they are limited by their side effect profile. Furthermore, their efficacy appears to be greater in cancers with a high mutational burden. We present two female patients with bilateral reactive dacryoadenitis secondary to ICPi therapy, a finding that to the best of our knowledge was not previously described in the literature.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"335-339"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141898646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}