Gabriella Schmuter, Richard N Polo, Shanlee Stevens, Celestine H Gregerson, Allison Coombs, Kevin Heinze, Abtin Tabaee, Theodore H Schwartz, Gary J Lelli, Kyle J Godfrey
{"title":"Orbital apex-like syndrome with multiple cranial neuropathies secondary to chronic inflammatory demyelinating polyneuropathy.","authors":"Gabriella Schmuter, Richard N Polo, Shanlee Stevens, Celestine H Gregerson, Allison Coombs, Kevin Heinze, Abtin Tabaee, Theodore H Schwartz, Gary J Lelli, Kyle J Godfrey","doi":"10.1080/01676830.2025.2562356","DOIUrl":"https://doi.org/10.1080/01676830.2025.2562356","url":null,"abstract":"<p><p>Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder characterized by progressive demyelination of peripheral nerves, leading to motor and sensory deficits. Ophthalmologic involvement, though uncommon, may manifest as ophthalmoplegia, papilledema, optic neuropathy, or proptosis. This report presents a 49-year-old male with refractory CIDP and extensive orbital involvement, including bilateral proptosis and enlargement of multiple cranial nerves, resulting in an orbital apex-like syndrome and compressive optic neuropathy. The patient's condition was refractory to medical management with intravenous immunoglobulin, corticosteroids, plasma exchange, and rituximab, and he ultimately required surgical decompression. Improvement in visual symptoms was noted in the early post-operative period. This case highlights the importance of a multidisciplinary approach in the management of complex CIDP cases with significant cranial nerve involvement and underscores the need for further research into its pathophysiology and treatment optimization.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1-6"},"PeriodicalIF":0.8,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145151037","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":"Single staged transorbital neuroendoscopic (TONES) approach of managing pulsatile proptosis using patient specific implant (PSI) in orbitopalpebral neurofibromatosis.","authors":"Prerna Sinha, Sumer Doctor, Tarjani Dave","doi":"10.1080/01676830.2025.2560631","DOIUrl":"https://doi.org/10.1080/01676830.2025.2560631","url":null,"abstract":"<p><p>Sphenoid wing dysplasia and plexiform neurofibromas are among the diagnostic criteria for neurofibromatosis 1 (NF1), an autosomal dominant neurocutaneous disorder. It has been called by different names based on the involved anatomic areas such as orbito-palpebral, orbito-temporal, and orbito-facial plexiform neurofibromatosis. Management of the deformity in plexiform neurofibromatosis is challenging given the ill-defined, infiltrative, and vascular nature of the lesion, associated with a defect in the greater wing of sphenoid, resulting in pulsatile proptosis. A staged multidisciplinary approach has been described in literature to correct the bony dysplasia followed by correction of the soft tissue components. Craniofacial bony deformities have been mostly described to be managed via craniotomy approach by neurosurgeons whereas the periorbital debulking of tumor and correction of canthal dystopia and blepharoptosis is done by ophthalmic plastic surgeons at a later stage. Bony defects have been traditionally managed with the help of bone grafts or titanium mesh or a combination of both, but, more recently, the use of patient specific implants has also been described which offer bespoke reconstruction. A single staged transorbital approach to reconstruct the skull base defect and orbital aperture along with palpebral debulking and levator resection has not been described till date to the best of the authors' knowledge. Here, the authors describe a case of orbitopalpebral plexiform neurofibromatosis with pulsatile proptosis managed via a single staged transorbital approach using computer-assisted 3 dimensional (3D) virtual surgical planning.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1-7"},"PeriodicalIF":0.8,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145114642","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}
Nkechi N Nwabueze, James O Robbins, Jason A Liss, Christopher R Dermarkarian
{"title":"Posterior scleritis mimicking orbital cellulitis with an intraconal abscess.","authors":"Nkechi N Nwabueze, James O Robbins, Jason A Liss, Christopher R Dermarkarian","doi":"10.1080/01676830.2025.2556434","DOIUrl":"10.1080/01676830.2025.2556434","url":null,"abstract":"","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1"},"PeriodicalIF":0.8,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145076464","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}
Mohadeseh Feizi, Alireza Amirabadi, Amir Arabi, Amir A Azari, Amirreza Veisi
{"title":"Fixation-free lateral orbitotomy using the periosteal covering technique for orbital tumor excision.","authors":"Mohadeseh Feizi, Alireza Amirabadi, Amir Arabi, Amir A Azari, Amirreza Veisi","doi":"10.1080/01676830.2025.2559742","DOIUrl":"https://doi.org/10.1080/01676830.2025.2559742","url":null,"abstract":"<p><strong>Purpose: </strong>To present outcomes of a lateral orbitotomy technique with periosteal coverage of the osteotomized segment instead of traditional fixation with a plate and screws.</p><p><strong>Methods: </strong>This retrospective interventional case series presents the outcomes of lateral orbitotomy with periosteal coverage of the osteotomized segment. We included all patients who underwent the lateral orbitotomy technique for intraorbital tumor removal between January 2005 and January 2021. Data on demographics, presenting signs and symptoms, clinical and histological diagnoses, and intraoperative and postoperative complications were collected and analyzed.</p><p><strong>Results: </strong>A total of 40 patients were eligible for inclusion. The mean age was 38.8 ± 17.08 years (range: 6 months to 65 years), and the mean follow-up period was 46.73 ± 24.15 months (range: 12-91 months). In all patients, the mass was completely excised, and the lateral orbital wall was successfully replaced using the \"periosteal covering technique\" without the need for K-wires or mini-plates for fixation. Postoperatively, there were no cases of non-union or malunion. Rare skin scars and occasional pain or tenderness at the surgical site were noted, along with a non-visible but palpable small bony step-off at the orbital rim edge.</p><p><strong>Conclusions: </strong>The periosteal covering technique appears to be a safe, simple, and time-efficient method for replacing osteotomized segments in cases of lateral orbitotomy with osteotomy during orbital tumor excision.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1-7"},"PeriodicalIF":0.8,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145076350","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}
Yousef Sefau, Ernest Chan, Musbah Khalaff, Ahsen Hussain
{"title":"Implant extrusion after eye removal for endophthalmitis and panophthalmitis.","authors":"Yousef Sefau, Ernest Chan, Musbah Khalaff, Ahsen Hussain","doi":"10.1080/01676830.2025.2553661","DOIUrl":"https://doi.org/10.1080/01676830.2025.2553661","url":null,"abstract":"<p><strong>Purpose: </strong>To evaluate the rate of orbital implant extrusion and exposure following enucleation or evisceration in patients with endophthalmitis or panophthalmitis, and to assess the influence of infectious etiology, implant type, and surgical technique on extrusion risk.</p><p><strong>Methods: </strong>A systematic review was conducted using MEDLINE, CINAHL, Embase, and Scopus for studies published between January 1980 and December 2024. Studies were included if they evaluated implant extrusion or exposure following eye removal surgery in patients diagnosed with endophthalmitis or panophthalmitis. Fourteen retrospective cohort studies met the inclusion criteria.</p><p><strong>Results: </strong>Extrusion or exposure rates ranged from 0% to 53%. Pseudomonas aeruginosa was the most frequently implicated pathogen. Non-porous implants, especially silicone, were more commonly associated with extrusion, while porous implants, particularly hydroxyapatite, demonstrated lower complication rates. No clear difference was observed between evisceration and enucleation in terms of extrusion risk.</p><p><strong>Conclusions: </strong>Implant extrusion is a significant postoperative complication in the setting or endophthalmitis or panophthalmitis. Pseudomonas aeruginosa and the use of non-porous implants may increase extrusion risk. The use of porous implants and appropriate prophylactic antibiotics may be associated with lower risk of extrusion. Further prospective studies are required to standardize risk assessment and prevention strategies.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1-7"},"PeriodicalIF":0.8,"publicationDate":"2025-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145076409","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}
Buse Guneri Beser, Sarinee Juntipwong, Mehriban Alizada, Victor M Elner, Hakan Demirci
{"title":"A complex case of recurrent bilateral alternating orbital inflammation associated to VEXAS syndrome.","authors":"Buse Guneri Beser, Sarinee Juntipwong, Mehriban Alizada, Victor M Elner, Hakan Demirci","doi":"10.1080/01676830.2025.2554893","DOIUrl":"https://doi.org/10.1080/01676830.2025.2554893","url":null,"abstract":"<p><p>VEXAS syndrome is a rare disorder marked by systemic inflammation and blood disorders, caused by somatic mutations in the <i>UBA1</i> gene of hematopoietic stem cells. Ocular manifestations are common in VEXAS syndrome. This study reports a 63-year-old male presenting with recurrent periorbital and orbital inflammation, dacryoadenitis, and orbital myositis. He first developed orbital inflammation in the right orbit, later in the left orbit. He was treated with systemic corticosteroid therapy. During a 2-year follow-up, he developed multi-organ inflammatory disorder and recurrence of orbital inflammation whenever the systemic corticosteroid dose fell below 20 mg/day. Ultimately, he was diagnosed with VEXAS syndrome. Identification of VEXAS syndrome in elder male patients with orbital inflammation is essential for tailored management. Treatment typically involves corticosteroids and, in refractory cases, Janus kinase inhibitors like ruxolitinib or IL-6 inhibitors. This case highlights the importance of considering VEXAS syndrome in the differential diagnosis of orbital inflammation, particularly in elderly males with concurrent inflammatory and hematologic abnormalities.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1-5"},"PeriodicalIF":0.8,"publicationDate":"2025-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145015226","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":"A giant dermoid cyst of the upper eyelid mimicking a lipoma in an elderly patient: a rare presentation without rupture or inflammation.","authors":"Onur Özalp, Mehmet Serhat Mangan, Ceyhun Arici","doi":"10.1080/01676830.2025.2551621","DOIUrl":"https://doi.org/10.1080/01676830.2025.2551621","url":null,"abstract":"","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1"},"PeriodicalIF":0.8,"publicationDate":"2025-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145001649","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}
Mumta Kanda, Nirodha Jayawickrema, Indran Davagnanam, Emma Samia-Aly, Tarang Gupta, Claire Daniel
{"title":"Cemiplimab in advanced periocular conjunctival and cutaneous squamous cell carcinoma: a United Kingdom case series.","authors":"Mumta Kanda, Nirodha Jayawickrema, Indran Davagnanam, Emma Samia-Aly, Tarang Gupta, Claire Daniel","doi":"10.1080/01676830.2025.2551617","DOIUrl":"10.1080/01676830.2025.2551617","url":null,"abstract":"<p><strong>Purpose: </strong>To evaluate the real-world efficacy, safety, and outcomes of cemiplimab for advanced periocular conjunctival and cutaneous squamous cell carcinoma (SCC).</p><p><strong>Methods: </strong>Retrospective case series of adults treated with cemiplimab for periocular conjunctival or cutaneous SCC at Moorfields Eye Hospital (2021-2025).</p><p><strong>Results: </strong>We included 9 patients (mean age 66, range 43-79; 56% female; 78% Caucasian). Prior treatments included surgery (44%), radiotherapy (22%), and one exenteration. Tumour origin was conjunctival (56%), cutaneous (33%), or unknown (11%). 8 (89%) showed orbital invasion, including 2 with intracranial spread. Cemiplimab was indicated when surgery was refused or as palliative treatment in cases not amenable to localized treatment. Mean cemiplimab duration was 12.3 months (range 2.1-36.4). Immune-related adverse events included grade 2 hypothyroidism (<i>n</i> = 2, continued therapy) and grade 2 hepatitis (<i>n</i> = 1, discontinued). Complete and partial responses were seen in 22% and 33%, respectively; 44% progressed. No significant difference in response was observed between conjunctival and cutaneous SCC (<i>p</i> = 1.00). 2 patients (22%) underwent exenteration after starting treatment. Average total follow-up was 50.8 months (range 17.4-130.6). One recurrence occurred 12 months after treatment cessation; no new metastases were reported during follow-up. Two patients died from unrelated causes.</p><p><strong>Conclusion: </strong>To our knowledge, this is the largest single case series of cemiplimab used in conjunctival SCC. Our results underscore the potential of cemiplimab as globe-sparing and vision-preserving treatment in advanced periocular conjunctival and cutaneous SCC, including more aggressive and complex cases with intracranial involvement or perineural spread, both as primary treatment and after previous surgery in recurrent cases.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1-11"},"PeriodicalIF":0.8,"publicationDate":"2025-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144974042","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}
Troy Karanfilian, Katrice M Karanfilian, Javiera Saavedra-Nazer, Omar P Sangueza, Angela Niehaus
{"title":"Keratoacanthoma of the conjunctiva.","authors":"Troy Karanfilian, Katrice M Karanfilian, Javiera Saavedra-Nazer, Omar P Sangueza, Angela Niehaus","doi":"10.1080/01676830.2025.2551619","DOIUrl":"https://doi.org/10.1080/01676830.2025.2551619","url":null,"abstract":"","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1"},"PeriodicalIF":0.8,"publicationDate":"2025-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144973998","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}
George A Villatoro, Taimur Siddiqui, Michael T Yen
{"title":"Manifestation of a subclinical orbital vascular malformation after sclerotherapy: tip of the iceberg.","authors":"George A Villatoro, Taimur Siddiqui, Michael T Yen","doi":"10.1080/01676830.2025.2551620","DOIUrl":"https://doi.org/10.1080/01676830.2025.2551620","url":null,"abstract":"","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1"},"PeriodicalIF":0.8,"publicationDate":"2025-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144974001","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}