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Introduction: Solitary fibrous tumor (SFT) is a rare borderline mesenchymal tumor typically arising in the pleura and involving the orbit as its most common extra-pleural location., Case Description: We herein describe two cases of orbital SFT arising in both a 69-year-old woman presenting with progressive proptosis of the left eye and a 49-year-old woman presenting with binocular diplopia. The diagnoses relied on histopathological analysis of biopsy samples. Because of the poor local prognosis, we decided to perform an orbital exenteration in the first case. In the second case, a complete resection surgery was performed., Conclusion: SFT presents classics histological features and immunohistochemical markers that are essential to the diagnosis. SFT is classified as a benign tumor, but in some cases, it can show aggressive behavior with the potential for local tissue invasion and, more scarcely, distant metastasis. This is why complete resection remains the treatment of choice for SFT., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Background: Thrombosis of the superior ophthalmic vein (SOV) is rare. It can present with heterogeneous symptoms and requires a careful diagnostic work-up., Methods: This article reports on a case series of three female patients with thrombosis of the SOV and performed an extensive review of the existing literature in PubMed., Results: All three patients showed different leading symptoms but all had a triad of restricted eye motility, exophthalmos and a difference in intraocular pressure with the higher pressure on the side of the protruded eyeball. The pathophysiological background differed in all cases: in the two first cases a pre-existing anticoagulation treatment had been paused shortly before the event. Cofactors were an intraorbital venous outflow obstruction due to an unclear mass in the orbital apex in one case and hypercoagulability due to a malignant disease in the other case. In the third case, there was a cavernous sinus fistula., Conclusion: The diagnosis should be considered not only in patients with dilated vessels of the anterior segment but also in unclear cases of exophthalmos or unilateral elevated intraocular pressure (IOP)., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

In this study, we aimed to present a rare case of pulsatile proptosis due to sphenoid wing dysplasia without the features of neurofibromatosis type 1 (NF1). A 17-year-old male patient presented with swelling in the superotemporal region of the right eye. Physical examination revealed facial asymmetry with a pulsatile, ill-defined, soft lesion with in the superotemporal region of the right orbit associated with pulsatile proptosis, downward dystopia, and hypotropia. Computer tomography imaging to establish a differential diagnosis showed temporal lobe herniation secondary to sphenoid wing dysplasia. The patient was assessed for NF1, which is most commonly associated with sphenoid wing dysplasia, but no evidence supporting the diagnosis was found. Patients presenting with proptosis should be carefully examined for pulsation and murmurs, and a trauma history should be investigated. Radiological imaging should be used to facilitate the differential diagnosis, and the current clinical condition should be managed with a multidisciplinary approach., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors., (©Copyright 2024 by the Turkish Ophthalmological Association / Turkish Journal of Ophthalmology published by Galenos Publishing House.)

A 68-year-old male presented with a one-month history of progressive proptosis and vision loss in the left eye. Examination of the left eye showed visual acuity of NLP, marked relative proptosis of 10 mm, and complete external ophthalmoplegia. CT orbits showed an extensive left orbital lesion with proptosis. Urgent orbital biopsy was undertaken. Intraoperatively, the patient developed new atrial flutter and fever. Bloodwork revealed metabolic derangements suggestive of tumor lysis syndrome. Systemic evaluation revealed a large tumor burden involving the retroperitoneal space. Histopathology of the orbital specimen showed non-germinal center diffuse large B-cell lymphoma. The patient passed away 3 days postoperatively due to rapidly progressive multisystem organ failure. Our case demonstrates an unusually aggressive presentation of DLBCL in which orbital mass was the first presentation of spontaneous tumor lysis syndrome owing to large systemic tumor burden.

Congenital opticmeningoceles were recently described as intraorbital optic nerve cystic lesions, in which neural tissue is found to be part of the lining of the lesion. We describe the case of an eleven-month-old patient with a right opticmeningocele, followed through seven years. He presented with proptosis and hypotropia since birth and imaging exams disclosed an intraconal cystic lesion. A needle puncture revealed a fluid characteristic of cerebrospinal fluid. Surgical drainage performed through a lid crease approach resulted in recurrence of the proptosis. Surgical excision of the capsule finally was definitive, and pathology revealed an epithelial-lined capsule. Eight months after the capsule excision, a strabismus surgery was performed to improve the right hypotropia. Although visual prognosis of opticmeningoceles is poor, a good cosmetic result can be obtained with a multidisciplinary approach involving orbit and strabismus surgeons. The complete capsule excision is believed to be the preferable surgical option for permanently treating the proptosis once needle puncture and cyst drainage are associated with recurrence. The orbital access for approaching the lesion depends on the location of the cyst and should provide wide exposure to avoid new surgical procedures in young patients.

Competing Interests: The authors have no financial or conflicts of interest to disclose.

This study reported a case of an arachnoid cyst of the sphenoid bone causing orbital signs and symptoms in a 58-year-old man with progressive proptosis and nonspecific discomfort in the OS. Orbital MRI showed a 3-cm homogeneous cyst within the left greater wing of the sphenoid bone. To the best of our knowledge, this is the first report of an intradiploic arachnoid cyst in the sphenoid bone with atypical radiological features, causing clinical symptoms, and managed through an eyelid approach, achieving a complete resolution with no complications., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Background: Posterior movement of ocular tissue secondary to orbital cavernous venous malformation shrinkage from fractionated stereotactic radiotherapy can allow healthy structures to move into the radiation field during treatment. This may carry an increased risk of radiation-induced retinopathy., Methods: We present a case of a young female whose radiotherapy treatment for an orbital cavernous venous malformation resulted in a 3 mm reduction in proptosis and subsequent retinopathy., Results: The severity of the patient's radiation-induced radiotherapy exceeded expectations. The venous malformation shrinkage during treatment and ensuing posterior movement of the globe suggested an increased involvement of ocular tissue in the radiation field, prompting consideration of interval neuroimaging and tumour mapping., Conclusions: We describe and suggest a protocol of onboard neuroimaging during the radiation therapy course to better target tumour volumes and minimise collateral tissue damage. To our knowledge, this has not been previously described in the ophthalmic literature., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

A 26-year-old male with a history of orbital lymphangioma and compressive optic neuropathy presented with recurrent proptosis in the OS. After examination and imaging, a left orbital lymphatic malformation and a new subcutaneous temporal-parietal vasculo-lymphatic malformation were diagnosed. The patient underwent a bleomycin injection for the orbital malformation and an excisional biopsy for the temporal lesion, leading to symptom resolution. Recurrence of lymphatic malformations and hemorrhage typically occurs at the same site, here we report a patient with the appearance of a new site lesion with orbital recurrence., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

The purpose of this article is to report a rare case of isolated superior ophthalmic vein thrombosis. A 74-year-old female presented to the emergency department with a sudden onset of eye pain and bulging. Ophthalmological examination was remarkable for proptosis and ptosis with chemosis of the OS. Neuroimaging demonstrated an isolated superior ophthalmic vein thrombosis secondary to presumed thrombosis of the superior vein varix. Hypercoagulable, infectious, and autoimmune lab workups were unremarkable. The patient was initiated on anticoagulation with the eventual resolution of her symptoms. Isolated superior ophthalmic vein thrombosis is an uncommon diagnosis that requires urgent evaluation to prevent vision loss. Risk factors are multifactorial with infectious being the most common etiology. Our case is unique in that there was no identifiable risk factor., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

A 37-year-old, previously healthy woman presented during her first trimester of pregnancy with a two-week history of rapidly progressive proptosis in the left eye. Clinical examination revealed limited left supraduction and diplopia in upward gaze. Orbital magnetic resonance imaging showed a medial orbital mass adjacent to the globe with secondary proptosis. Pathologic examination of a biopsied specimen of the orbital mass and subsequent immunophenotyping by flow cytometry revealed an extranodal marginal zone B-cell lymphoma. Clinical and histological features as well as a review of the literature are described.

Purpose: To determine the recurrence and reactivation rates after teprotumumab therapy for active thyroid eye disease., Design: Retrospective consecutive case series., Methods: This was a study of all patients followed for active thyroid eye disease at the Cole Eye Institute, Cleveland Clinic, treated with teprotumumab between May 2020 and May 2021. Patients with less than 6 months follow-up after completion of infusions were excluded. The primary outcome measure was reactivation, defined as a regression in proptosis (increase of ≥2 mm in either eye and to within ≤2 mm of pre-treatment level and Clinical Activity Score [CAS] worsening of 2 points or greater). Secondary outcome was diplopia response., Results: A total of 21 patients were included in the study. The average long-term improvement in proptosis in the eye with more proptosis after teprotumumab was 1.57mm (range, -3 to 4 mm). Of the 17 initial responders, there were 8 reactivations (47%) and 2 isolated proptosis regressions (12%); Overall, 7 of 21 patients (33%) responded throughout the study period. Average time to regression was 12.25 months (range, 2-22.5 months). There was no statistically significant change in diplopia at final visit in any subgroup (P = 0.68 to >.99)., Conclusions: At most, 33% of patients demonstrate continued response 2 years after teprotumumab treatment. The proptosis and CAS regression occurs in the setting of disease reactivation in 80% of regressions. Teprotumumab treatment appears to offer minimal long-term improvement in diplopia., (Copyright © 2023. Published by Elsevier Inc.)

A 70-year-old male presented with diplopia and painless proptosis of the left eye for 5 months. Examination showed 6 mm of axial proptosis and restriction of supraduction, abduction and adduction, and mild limitation of infraduction of the left eye. Magnetic resonance imaging demonstrated a large, moderately well-circumscribed intraconal mass in the left lateral orbit, and excisional biopsy was performed. Histopathologic features of mixed fibrous and myxoid areas in a whorl-like pattern and immunohistochemical staining for MUC4 confirmed the diagnosis of low-grade fibromyxoid sarcoma (LGFMS). Next-generation sequencing revealed genetic fusion of EWSR1-CREB3L1. LGFMS is an extremely rare neoplasm with only two prior documented cases of orbital involvement. Here, we report the third case of orbital LGFMS.

Purpose: To clarify the clinical, diagnostic, and treatment characteristics of orbital liposarcoma., Design: Retrospective observational case series., Methods: A review was performed of electronic medical records, histopathology, radiological images, and follow-up information for 21 patients with orbital liposarcoma., Results: The predominant clinical manifestation of this disease was painless exophthalmos. The most frequently encountered pathological types were well-differentiated and myxoid liposarcoma. Preoperative radiological images from 15 patients showed that orbital liposarcoma initially developed in extraocular muscle in 9 patients. Furthermore, all well-differentiated liposarcomas exhibited the radiographic characteristics of irregular and ill-defined adipose tissue, whereas only 12.5% of myxoid liposarcomas had the imaging characteristics. For the patients who exclusively underwent surgery, all of those with subtotal excisions experienced recurrence, 63.6% of marginal excisions recurred, and 50% of wide excisions resulted in recurrence. However, none of the patients who underwent marginal excisions or wide excisions combined with adjuvant radiotherapy exhibited recurrence. The analysis of magnetic resonance imaging findings in 3 patients who underwent neoadjuvant radiotherapy revealed that the tumor size remained stable in a patient with well-differentiated liposarcoma, whereas another patient with the same type of tumor exhibited a gradual increase in size. Conversely, a patient with myxoid liposarcoma experienced a significant reduction in tumor size following neoadjuvant radiotherapy., Conclusion: Orbital liposarcoma demonstrated a propensity for developing in the extraocular muscle. The radiological images of orbital well-differentiated liposarcomas were characterized by irregular and ill-defined adipose tissue. Surgery combined with radiotherapy demonstrated potential in reducing recurrence rates. Notably, orbital myxoid liposarcoma exhibited greater sensitivity to radiotherapy compared to well-differentiated liposarcoma., (Copyright © 2024 Elsevier Inc. All rights reserved.)

A 5-year-old girl presented with a 6-month history of strabismus and painless left proptosis. Magnetic resonance imaging revealed a homogenously enhancing, anterior, superomedial, left orbital mass. Excisional biopsy established the diagnosis of orbital glomangiopericytoma. Glomangiopericytoma of soft tissue is a perivascular myoid neoplasm with hemangiocytoma-like vascular channels that has overlapping features between glomus tumor and myopericytoma. To the authors' knowledge, glomangiopericytoma of the orbit has not previously been reported.

A 33-year-old lady was referred to our clinic with a history of left upper eyelid swelling and difficulty to open her left eye for more than 4 years. Her globe was in infero-nasal displacement, and ocular movement was not full, with restriction to every direction. No clinical sign including eye redness, pain or visual loss was found on examination. Exophthalmometry confirmed 2 mm of left-sided proptosis. Computed tomography (CT) imaging of the orbit obtained in axial and coronal planes depicted an irregular and heterogeneous soft-tissue density mass in the left lacrimal gland, measuring 25 × 17 mm. Magnetic resonance imaging (MRI) revealed the mass of slightly longer T1 and slightly longer/shorter T2 signal, and Gd-enhanced MRI showed a significant enhancement. The tumor was first presumably diagnosed of pleomorphic adenomas. A surgery was applied to remove the tumor completely. Histopathological evaluation led to the diagnosis of PEComa.

Abstract: Carotid-cavernous fistulas are rare entity with incidence of less than 1%, refers to abnormal connections between the carotid artery and cavernous sinus. Indirect types usually occur in elderly female patients and can resolve spontaneously with conservative management like external manual compression of the carotid artery. We report a case of 65-year-old female who presented with complaints of redness, proptosis, chemosis, headache and ophthalmoplegia in her right eye. Digital subtraction angiography revealed Barrow type B indirect carotid-cavernous fistulas. External manual carotid compression was done after which her symptoms improved significantly. Thus, indirect type carotid-cavernous fistulas can occur spontaneously and could be a sight threatening condition especially in elderly females but can resolve with conservative management.

Purpose: To compare outcomes of patients with thyroid eye disease treated with teprotumumab or orbital decompression, or both in sequence., Methods: Patients with thyroid eye disease and treated with decompression, teprotumumab, or both were included. Four groups were defined: decompression only, teprotumumab only, teprotumumab first with decompression later, and decompression first with teprotumumab later. The primary outcome was change in exophthalmometry. Secondary outcomes included change in extraocular muscle motility, strabismus, diplopia, and side effects., Results: One hundred and thirty-nine patients were included. The mean duration for early follow-up was 1.2 months for both decompression and teprotumumab groups. The mean late follow-up was 14.4 and 8.2 months for the decompression and teprotumumab groups respectively. Mean change in exophthalmometry was significantly greater for the decompression group (3.5 mm) compared with teprotumumab (2.0 mm) at late follow-up. Improvement in total extraocular muscle restriction was significantly greater in the teprotumumab group (14.7 degrees) than in the decompression group (2.6 degrees). The teprotumumab group had a significantly higher percentage of patients with diplopia score >1 at baseline and late follow-up ( p < 0.01) compared with the decompression group. Additional treatment with teprotumumab or decompression when previously treated with the opposite had similar proptosis reduction effect as that therapy alone., Conclusions: Surgical decompression has a greater proptosis reduction effect than teprotumumab, whereas teprotumumab better improves extraocular muscle motility. The addition of teprotumumab or decompression to a previous course of the opposite adds a similar effect to the supplemental treatment alone., Competing Interests: D.B.R. is a consultant for Horizon Therapeutics. The other authors have no conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Competing Interests: The authors declare that they have no conflict of interest.

Purpose: Patients with thyroid eye disease (TED) can present with asymmetric disease. The purpose of this study was to evaluate the prevalence of asymmetric TED in an Australian cohort and investigate clinical, biochemical, and radiological associations of asymmetric TED., Methods: This was a retrospective study of patients with TED who underwent Hertel exophthalmometry and orbital computed tomography (CT) scans. Asymmetry was defined as a difference in the globe protrusion of ≥ 3 mm using Hertel exophthalmometry. Data was collected on patient demographics, clinical disease presentation, thyroid function tests and antibody levels. Muscles volumes were determined by manually segmenting the extraocular muscles on CT scans using a commercially available software., Results: 172 orbits from 86 patients were included in the study. 28 (33%) patients had asymmetric TED. No significant differences were observed in age, gender, thyroid hormone status, anti-thyroid peroxidase levels, thyroid stimulating hormone receptor antibodies, disease activity status, or dysthyroid optic neuropathy between the asymmetric and non-asymmetric groups. The extraocular muscle volumes were significantly higher in the asymmetric orbit compared to its contralateral orbit., Conclusion: Asymmetric TED is a reasonably common occurrence in the course of TED. It is associated with higher extraocular muscle volumes, suggesting muscle enlargement as one of the underlying contributors to asymmetric proptosis. Thyroid eye disease should be considered in the differential of asymmetric orbital inflammatory disease., (© 2024. Crown.)

A 71-year-old male presented with painless and slowly progressive proptosis of the right eye. The patient had no history of cancer and was in good health. Orbital magnetic resonance imaging showed a well-demarcated, slightly lobulated intraconal orbital mass hypo-isointense on T1- weighted and isointense on T2-weighted images, demonstrating contrast enhancement in the right orbit. The lesion had a central cavitary region. An anterior orbitotomy with lower lid crease incision was performed and the lesion was excised in toto. Histopathologic examination revealed diffuse infiltration of monomorphic population of small lymphoid cells. Immunohistochemical staining demonstrated diffuse and strong positivity for CD20, CD5, and cyclin D1. Histopathological and immunohistochemical findings were consistent with mantle cell lymphoma. Positron emission tomography-computed tomography did not show systemic involvement. The patient underwent orbital external beam radiotherapy (36 Gy). There was no recurrence and/or systemic involvement at 2.5 years follow-up.

Secondary metastasis of rhabdomyosarcoma (RMS) to the orbit from a distant primary site is extremely rare in adults. In this article, we describe the case of a 24-year-old male presenting with proptosis, diplopia, and headaches concurrently with a histologically confirmed diagnosis of PAX3-FOXO1 positive paraspinal alveolar rhabdomyosarcoma. An orbital MRI revealed a fusiform mass arising from the inferior rectrus, displaying necrotic and irregular morphology consistent with malignancy. The patient is currently undergoing intensive chemotherapy. The objective of this case report is to highlight the rarity of an extraocular metastasis of RMS in an adult patient, alongside the importance of considering metastatic disease in a patient with fulminant unilateral proptosis.

Purpose: This study analyzed the degree and timing of proptosis regression after teprotumumab therapy., Methods: A retrospective study of all patients who completed 8 teprotumumab infusions at 1 institution from January 1, 2020 to December 31, 2022. Change in proptosis was assessed in millimeters and percentages compared with immediate post-treatment and pretreatment proptosis., Results: Of 119 patients with post-treatment data (mean follow-up 10.56 months, range: 3.05-25.08), 208 (87.39%) eyes of 110 patients had initial proptosis improvement. Of the 78 patients with multiple follow-up visits, 102 (65.38%) eyes of 59 patients had proptosis regression averaging 12.78% (range: 1.85-58.82%) compared with immediately post-treatment or 2.43 mm (0.5-10.0 mm). Eight (7.84%) eyes had initial documentation of regression more than 1 year after treatment, 40 (39.22%) between 6 months and 1 year, and 54 (52.94%) eyes within 6 months with 25 (46.30%) of these continuing to worsen at subsequent follow-up. Forty (25.64%) eyes of 24 patients had more proptosis at most recent follow-up than before teprotumumab, with an average regression of 1.53 mm (0.5-4.0 mm) or 7.74% (1.85-20.69%) of pretreatment proptosis. In comparison, 99 (63.46%) eyes of 54 patients maintained improvement, with reduction averaging 3.13 mm (0.5-11.0 mm) or 13.19% (1.92-41.67%) of pretreatment proptosis ( p < 0.001)., Conclusions: Two-thirds of eyes had regression despite initial teprotumumab response, typically within 1 year of treatment, with ongoing worsening over time. Most patients maintained some proptosis reduction compared with before treatment despite regression, although 25% were worse than pretreatment. The occurrence of regression was independent of the pretreatment duration of clinical thyroid eye disease. Overall, compared with preteprotumumab, there was a greater amount of improvement than regression at most recent follow-up., Competing Interests: M.K.Y.: is a researcher for Sling and Viridian; S.K.F.: is a consultant for ASPEN/Third Rock Horizon, Immunovant, Lassen, Medtronic, Poriferous, Sling, Viridian, and WL Gore. Other authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: This study aims to evaluate the long-term outcomes of inferomedial orbital wall decompression (IMOD) in Graves' ophthalmopathy (GO)., Methods: A retrospective review of 422 eyes of 226 patients with GO-related cosmetically disfiguring proptosis (COS), dysthyroid optic neuropathy (DON), or exposure keratopathy (EXP) who received IMOD from 1989 to 2020 was conducted. Hertel value (HE) and corrected visual acuity (CVA) were evaluated at baseline and regularly thereafter. Proptosis recurrence, diplopia, and adjuvant surgeries were assessed. Complete success was defined as proptosis reduction over 2 mm without recurrence and improved diplopia. Partial success was defined as proptosis reduction without recurrence but with persistent or new-onset diplopia, and failure as proptosis recurrence., Results: After follow-up for 40.1 ± 39.6 months (range, 6-239 months), 236 eyes (55.9%) achieved complete success, 175 eyes (41.5%) achieved partial success, and 11 eyes (2.6%) had failure. Significant CVA improvement was observed in both DON and COS groups (P < 0.001 and P = 0.045, respectively). Average proptosis reduction was 5.2 ± 2.2 mm, with a significant increase of 0.5 mm after long-term follow-up (P < 0.001). Incidence of proptosis recurrence was 2.6%. Surgical success rate of COS group was positively correlated with preoperative HE., Conclusion: IMOD demonstrated excellent success rate in all three subgroups with a low proptosis recurrence rate and improved visual outcome in both DON and COS groups. Delayed proptosis reduction effect of IMOD may result in delayed-onset asymmetry, especially in the unilateral group., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Purpose: Rehabilitative orbital decompression treats disfiguring exophthalmos in patients with Graves' orbitopathy (GO). This study aimed to identify risk factors associated with the postoperative recurrence of proptosis after orbital decompression., Design: Retrospective, case-control study., Methods: This retrospective review included patients with GO who underwent rehabilitative orbital decompression for disfiguring proptosis in an inactive state with a low clinical activity score (0-2) between January 2017 and December 2020 by a single surgeon. Exophthalmos was measured using a Hertel exophthalmometer, and recurrence was defined as an increase of 2 mm or more after decompression during the follow-up period. The association between preoperative variables and proptosis recurrence was analyzed using multivariable logistic regression., Results: Of the total 217 patients, 11 (5.1%) developed recurrence of proptosis during the follow-up period (range, 3-30; mean, 15.6 months). Univariate logistic regression analysis identified thyroid-stimulating hormone receptor antibody (TRAb) and thyroid-stimulating immunoglobulin (TSI) as significant factors for recurrence, with age, sex, smoking, disease duration, orbital radiotherapy, and total thyroidectomy history being nonsignificant. TRAb remained significant in a multivariate logistic regression analysis (odds ratio, 1.06; P = .014). Receiver operating characteristic curve analysis revealed an area under the curve of 0.86 with a sensitivity of 90.9% and specificity of 82.0% at a TRAb level of 7.96 IU/L., Conclusion: Preoperative TRAb and TSI are valuable markers to predict proptosis recurrence after orbital decompression. These results may help surgeons to decide the optimal timing for orbital decompression to lessen the risk of postoperative recurrence of proptosis., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Proptosis is a frequent presenting symptom/sign of many paediatric malignancies. Acute-onset proptosis is an ophthalmic emergency that can endanger vision if not treated promptly. Appropriate treatment must be instituted only after investigating for the underlying aetiology. Here, we report a developmentally delayed boy in middle childhood who presented with recent onset bilateral proptosis. Clinical examination followed by radiological evaluation suggested scurvy to be the underlying cause and vitamin C supplementation led to prompt reversal of proptosis. The relevant literature has been reviewed and presented here to apprise the paediatric oncologists about this rare but easily treatable cause of proptosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

A healthy 32-year-old woman presented with the acute onset of left sided eye pain, upper eyelid fullness, and binocular diplopia during light weightlifting. Examination elevated intraocular pressure, proptosis, upper eyelid ptosis, and motility deficits. CT demonstrated a well-circumscribed, homogeneous-appearing extraconal mass in the superior left orbit. The patient underwent an urgent orbitotomy with the excision of a hemorrhagic mass. Histopathology showed a glomus tumor with atypical features and hemorrhagic infarction, best classified as having uncertain malignant potential. A B-Raf proto-oncogene V600E mutation was detected with immunohistochemistry, which suggests a more aggressive tumor behavior yet presents an opportunity for targeted primary or adjunctive therapy. This is the first reported case of a B-Raf proto-oncogene-mutant atypical glomus tumor arising in the orbit., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: The purpose of this study was to analyze Rundle's original data and subsequent articles on Graves orbitopathy (GO) natural history., Methods: Rundle's texts were analyzed qualitatively and quantitatively. Serial measurements were plotted and fitted with different functions. Subsequent articles in the English literature on the natural history of GO were also analyzed., Results: Different functions such as simple linear regressions, parabolic, saturating exponential growth, and exponential decay functions were well fitted for Rundle's data on measurements of proptosis and supraduction along time. The few quantitative data of the same variables post-Rundle were also well-fitted with various functions., Conclusion: Rundle described in his articles, from 1945 to 1957, 2 phases of ocular changes in GO: dynamic and static. However, he did not mention the pathophysiology of these phases nor used the terms inflammatory and cicatricial. Actually, most of his observations and the subsequent data in the literature on proptosis and supraduction did not obey the biphasic pattern of the so-called Rundle's curve., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To determine the accuracy of manual compass measurement and trigonometric determination of proptosis (MCMATDP)., Methods: This agreement study included 120 eyes without eye diseases or injury of 60 patients who visited the ophthalmic clinic of Peking University Shenzhen Hospital from February 2020 to June 2020. The absolute values of proptosis were measured by MCMATDP and computed tomography (CT). The differences between the two methods were shown by Bland-Altman plot., Results: The cohort comprised 25 males and 35 females (average age 38.3 years). The absolute value of proptosis measured by CT was correlated with the MCMATDP. Further analysis showed that a 95% limit of agreement (LoA) was - 0.53 to 0.60 mm in the right eye and - 0.46 to 0.55 mm in the left eye between CT and MCMATDP. In addition, the 95% LoA was - 0.49 to 0.60 mm in both eyes between the two methods. All points were < 5% in Bland-Altman plots., Conclusions: Compared to CT, MCMATDP is rather consistent in proptosis measurement. The new method is feasible in clinical practice when measuring proptosis. With the development of non-contact intelligent measurement software and the continuous improvement in measurement accuracy, a non-invasive, simple, and inexpensive measurement mode is true based on the theory of MCMATDP., (© 2023. The Author(s), under exclusive licence to Springer Nature B.V.)

A 2-year-old boy presented with left periorbital edema, proptosis, hyperglobus and esotropia. Imaging revealed an inferotemporal orbital mass with adjacent bony erosion. Histological evaluation of an orbital biopsy revealed B-cell acute lymphoblastic leukemia/lymphoma (B-ALL/BLL). The patient was subsequently treated with chemotherapy. Although orbital involvement in acute myelogenous leukemia has been well-described, orbital manifestations of B-ALL/BLL are uncommon, with only a limited number of previous reports in the literature.

A 31-year-old female presented to the clinic with a 2 year history of proptosis of the right eye. She was diagnosed with cavernous hemangioma during her initial ophthalmologic consult and was advised monitoring. However, after a considerable increase in proptosis, she underwent an excision biopsy of the right orbital mass. A diagnosis of Rosai Dorfman disease was made after the histopathology report revealed a fibrosclerotic tissue with histiocytic proliferation showing emperipolesis that is mixed with numerous small lymphocytes and plasma cells. Rosai Dorfman disease is a rare disease presenting with lymphadenopathy and sinus histiocytosis. Orbital involvement can be the principal manifestation with proptosis as the most common presentation. Resection of the orbital lesion helped in the resolution of the mild ophthalmic symptoms but since the disease has other systemic associations, a complete systemic workup should be done to monitor recurrence.

Aims: To evaluate presenting features of patients with orbital solitary fibrous tumours (SFTs), based on histological phenotype., Methods: A retrospective case-note review was performed for demographics and presenting features for patients with orbital SFTs. The tumours were classified as "Group IA" hypocellular SFT phenotype, "Group IB" haemangiopericytoma phenotype and low mitotic activity, and high-grade "Group II" haemangiopericytoma phenotype with high mitotic activity., Results: Sixty-four patients (34 female; 53%) presented at a mean age of 42.2 years (median 38; range 19-82), with Group II patients presenting at an older age (mean 53 years). Median symptom duration was 12 months for Groups IA and IB, compared to 4 months for Group II, the commonest symptoms being proptosis (53%), diplopia (41%), periorbital swelling (31%), and altered vision (19%). Mean LogMAR was 0.17 (median 0.0; range -0.2-4), and 14% had ipsilateral optic neuropathy, with no significant difference between the three groups. Non-axial displacement was noted in 69%, a palpable mass in 45%, and reduced eye movements in 59%; choroidal folds and optic disc swelling were recorded in 12% and 9%. SFTs were mostly extraconal (59%), within the superior and superonasal quadrants (44%), with an average estimated tumour volume of 4.9 ml (median 3.6; range 0.31-14.5 ml)., Conclusion: SFTs may present with impaired visual function (∼15%), fundal abnormalities (a fifth), globe displacement (two-thirds), and reduced ocular motility (over a half). High-grade tumours tend to present more than a decade later, with a shorter duration of symptoms., (© 2023. Crown.)

Importance: The current clinical criterion standard for measuring abnormal eyeball protrusion is still the historic Hertel exophthalmometer, which is prone to reading errors. Therefore, a smartphone application has been developed to measure exophthalmos., Objective: To evaluate a relatively simple noninvasive measurement method for exophthalmos using a smartphone., Design, Setting, and Participants: This cross-sectional study compared smartphone exophthalmometry with the existing reference standard, the Hertel exophthalmometer, or a professional high-resolution 3-dimensional scanner. Participants were patients with exophthalmos due to Graves orbitopathy and other intraorbital conditions and healthy volunteers who were recruited between June 2019 and January 2022 from the Department of Ophthalmology, University Hospital Zurich., Interventions: All participants were examined twice by 3 different operators using 3 different methods (smartphone, high-resolution scanner, or Hertel exophthalmometer) at an interval of a minimum of 2 weeks or after exophthalmos-changing treatment., Main Outcome Measures: Accuracy and precision, test-retest reliability, and interoperator reliability of eyeball protrusion measurements obtained with the smartphone compared to the Hertel exophthalmometer and the high-resolution face scanner., Results: Of 39 participants, 23 patients (median [IQR] age, 54 [44-59] years; 15 [65%] female and 8 [35%] male) showed a mean difference in eyeball protrusion of 3.3 mm and 16 healthy volunteers (median [IQR] age, 32 [30-37] years; 11 [69%] female and 5 [31%] male) of 0.8 mm without any significant difference between the 3 methods. Accuracy and precision agreement between exophthalmos measures with the smartphone and the Hertel exophthalmometer showed an intraclass correlation coefficient (ICC) of 0.89 (95% CI, 0.80-0.94) and 0.93 (95% CI, 0.83-0.97) for the high-resolution scanner. Interoperator agreement was highest for the high-resolution scanner (ICC, 0.99 [95% CI, 0.98-0.99]), followed by the smartphone (ICC, 0.95 [95% CI, 0.92-0.97]) and the Hertel exophthalmometer (ICC, 0.91 [95% CI, 0.85-0.95]). Test retest reliability was similarly high for the smartphone (ICC, 0.93 [95% CI, 0.82-0.95]), the Hertel exophthalmometer (ICC, 0.92 [95% CI, 0.83-0.96]), and the high-resolution scanner (ICC, 0.95 [95% CI, 0.89-0.97])., Conclusions and Relevance: The findings of this study demonstrate relatively high accuracy and precision, interoperator reliability, and test-retest reliability for smartphone exophthalmometry. These data support the use of a smartphone in place of a Hertel exophthalmometer for measuring exophthalmos in the future.

Background: Tentorial dural arteriovenous fistulas (TDAVFs) are abnormal shunts between meningeal arteries and the intradural venous system located in the tentorial dura mater, which typically manifest with haemorrhage or progressive neurological disorders. TDAVFs with pure ocular presentation have been rarely reported., Case Presentations: The case of a 56-year-old man presented with unilateral eye redness, proptosis and elevated intraocular pressure was reported herein, which was caused by a TDAVF. The fistula was fed by the left posterior cerebral artery and posterior meningeal artery. The drainage was into the basal vein and internal cerebral veins, which led the arterial blood flow forward to the left superior ophthalmic vein directly. The redundant blood flow caused the rise of episcleral venous pressure, leading to the clinical presentations. Gamma knife radiosurgery was performed then considering the delicate vascular structure and its deep location. The corkscrew hyperaemia was gradually alleviated after the surgery, but the intraocular pressure remained elevated at follow-ups., Conclusion: Dural arteriovenous fistulas which are not directly connected to cavernous sinus could cause ocular presentations like proptosis, eye redness and ocular hypertension., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Purpose: To report and investigate proptosis in a young girl with Noonan syndrome., Methods: Observational case report., Results: A 16-year-old girl affected by Noonan syndrome underwent a complete ophthalmological examination showing bilateral proptosis with hypofunction of lateral rectus and superior oblique muscles. Visual acuity, color discrimination and fundus examination were unremarkable. The orbital MRI showed bilateral proptosis and symmetrical enlargement of extraocular muscles, with bellies thickening and tendon sparing. The young patient also complained restrictive hypertrophic cardiomyopathy., Conclusions: Proptosis is an uncommon ocular manifestation of Noonan syndrome and its pathophysiology has never been clarified. The MRI evidence of extraocular muscles enlargement associated with hypertrophic cardiomyopathy, led us to hypothesize a common altered pathway beneath these features, more specifically the MAP kinase pathway, since extraocular and cardiac muscles share a mesenchymal embryological origin.

A 12-year-old Japanese male presented with a 2-month history of headache that was later on associated with diplopia, painless proptosis of the OS, and left ophthalmoplegia. Initial examination revealed a 7-mm OS protrusion, which worsened to 9 mm in less than a month. Preoperative visual acuity worsened from 1.0 to 0.2 with the development of left afferent pupillary defect. Left ocular motility was severely restricted in all directions. Magnetic resonance imaging showed two well-defined lesions adjacent to one another in the left orbit. The patient underwent surgical excision of the left orbital masses. Histopathology findings were consistent with solitary fibrous tumor of the orbit. Immunohistochemistry findings revealed CD34-negative but signal transducer and activator of transcription 6-positive for both specimens. The patient was monitored postoperatively and there was no recurrence of the tumor even after 6 months., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Malignant masses of the orbit include a large variety of neoplasms of epithelial or mesenchymal origin. The treatment of orbital malignancies is an essential interdisciplinary field of medicine that integrates ENT medicine, facial surgery, plastic surgery, neurosurgery, oncology and radiology.The main symptom of malignant orbital masses is the exophthalmos. A symptom that can help to differentiate a benign from a malignant orbital mass can be the pain. The main diagnostic tool is the MRI including new sequences like DWI and DCE.After presenting symptoms and diagnostic strategies of malignant orbital masses, this article starts with the description of malignant epithelial neoplasms of the lacrimal gland. Furthermore, it describes new insights in orbital lymphomas, followed by the discussion of semimalignant orbital masses. Last but not least the text deals with malignant neoplasms of the skin that can grow secondarily in the orbit. Finally, the manuscript discusses orbital metastases., Competing Interests: Erklärung zu finanziellen Interessen Forschungsförderung erhalten: nein; Honorar/geldwerten Vorteil für Referententätigkeit erhalten: nein; Bezahlter Berater/interner Schulungsreferent/Gehaltsempfänger: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an im Bereich der Medizin aktiven Firma: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an zu Sponsoren dieser Fortbildung bzw. durch die Fortbildung in ihren Geschäftsinteressen berührten Firma: nein. Erklärung zu nichtfinanziellen Interessen Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Purpose: The authors aim to describe the ophthalmologic manifestations of pediatric Erdheim-Chester disease (ECD)., Methods: The authors describe a novel case of ECD presenting as isolated bilateral proptosis in a child and provide a comprehensive review of the documented pediatric cases to observe overall trends and ophthalmic manifestations of disease. Twenty pediatric cases were identified in the literature., Results: The mean age at presentation was 9.6 years (1.8-17 years) with a mean time of symptom presentation to diagnosis of 1.6 years (0-6 years). Nine patients (45%) had ophthalmic involvement at diagnosis, 4 who presented with ophthalmic complaints: 3 with observable proptosis and 1 with diplopia. Other ophthalmic abnormalities included eyelid findings of a maculopapular rash with central atrophy on the eyelids and bilateral xanthelasmas, neuro-ophthalmologic findings of a right hemifacial palsy accompanied by bilateral optic atrophy and diplopia, and imaging findings of orbital bone and enhancing chiasmal lesions. No intraocular involvement was described, and visual acuity was not reported in most cases., Conclusions: Ophthalmic involvement occurs in almost half of documented pediatric cases. Typically presenting with other symptoms, the case highlights that isolated exophthalmos may be the only clinical sign, and ECD should be included in the differential diagnosis of bilateral exophthalmos in children. Ophthalmologists may be the first to evaluate these patients, and a high index of suspicion and an understanding of the varied clinical, radiographic, pathologic, and molecular findings are critical for prompt diagnosis and treatment of this unusual disease., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Primary yolk sac tumor of the orbit is a rare entity. Orbital involvement is usually seen in young children and proptosis is the commonest presentation. Aggressive orbital involvement and presentation as a fungating mass is rarely seen. We report a case of primary orbital yolk sac tumor with an aggressive presentation that responded well to systemic chemotherapy.

Burkitt lymphoma (BL) is an aggressive, rapidly growing B-cell non-Hodgkin lymphoma found predominantly in children and has three clinical subtypes. The sporadic subtype, seen in non-endemic areas, typically presents as an abdominal mass. Primary orbital involvement is rarely reported. We report two cases of sporadic orbital BL manifesting as unilateral rapidly progressive proptosis with orbit being the initial site of presentation. Following an incision biopsy, BL was confirmed on histopathology and immunohistochemistry. Both patients demonstrated a remarkable improvement with systemic chemotherapy. Burkitt lymphomas grow rapidly with the potential for vision loss. Albeit rare, clinicians should be aware of this entity as timely diagnosis and initiation with chemotherapy display a dramatic response.

Background: Lacrimal gland adenoma is a benign tumour of the lacrimal gland mostly involving the orbital part of the gland and composed of epithelial and myoepithelial components. It involves the third and fourth decade of life as a gradual painless enlargement of the lacrimal gland., Case: This is a case report of a 30-year-old female presenting with the forward bulging of the right eye causing the eye to be displaced medio-inferiorly over the course of one year. After careful clinical examination and MRI, a clinical diagnosis of lacrimal gland adenoma was established and was planned for right lateral orbitotomy (without marginotomy) via eyelid crease incision and transcutaneous-transseptal approach with complete excision of the tumour under general anaesthesia., Observations: This case was presented to the out patient department with mild painful non-axial proptosis of the right eye. After clinical examination, her MRI report showed a well-defined altered signal intensity enhancing lesion on the extraconal compartment of the right orbit in the antero-supero-lateral aspect. After complete surgical removal of the mass, it was sent for histopathological analysis and it confirmed it as a pleomorphic adenoma. The patient has been following up every 6 months for 2 years and is asymptomatic., Conclusion: Being the most prevalent lacrimal gland tumour, pleomorphic adenoma affects the unilateral lacrimal gland causing non-axial proptosis. Complete removal of mass has an excellent prognosis with complete resolution of symptoms., (© NEPjOPH.)

Objective: Granulomatosis with polyangiitis (GPA) is an autoimmune disorder characterized by necrotizing granulomatous inflammation of small and medium-sized vessels. This systematic review aimed to highlight the most common ophthalmic manifestations and to uncover their associations with antineutrophil cytoplasmic antibody (ANCA) positivity and the presence of granulomas. Methods: A literature search of PubMed, Web of Science, and Scopus electronic databases was performed from journal inception to March 21, 2021, for case reports and a series of ophthalmic GPAs. Cytoplasmic-ANCA (c-ANCA), perinuclear-ANCA (p-ANCA), and granulomas were analyzed against many ophthalmic signs and symptoms. 306 patients with GPA were retrospectively studied. Results: Granulomas were present in 47.7% of our sample, c-ANCA in 59.2%, and p-ANCA in 10.8%. Scleritis was significantly associated with higher odds for c-ANCA positivity. Eye discharge, episcleritis, proptosis, and central nervous system (CNS) involvement were each significantly associated with lower odds for c-ANCA positivity. Orbital mass was significantly associated with lower odds for p-ANCA positivity. CNS involvement was significantly associated with higher odds for p-ANCA positivity (OR:3.08, 95% CI:1.02, 9.36, p=0.047) and orbital mass was significantly associated with lower odds for p-ANCA positivity. Conclusions: We recommend that clinicians should consider ocular or orbital GPA in patients presenting with non-specific eye complaints, such as vision impairment, orbital mass, or proptosis, and obtain further assessments to determine the possible presence of granuloma, c-ANCA, or p-ANCA. Abbreviations: GPA = Granulomatosis with Polyangiitis, ANCA = antineutrophil cytoplasmic antibody, c-ANCA = cytoplasmic-ANCA, p-ANCA = perinuclear-ANCA, CNS = central nervous system, AAVs = ANCA-associated vasculitides, SD = standard deviation, GU = genitourinary, ENT = ear nose and throat, OR = odds ratio, CI = confidence interval., (#x00A9; The Authors.Romanian Society of Ophthalmology.)

Purpose: To assess the evolution of proptosis asymmetry during the active phase of bilateral thyroid eye disease (TED)., Methods: A retrospective study was conducted on patients with bilateral, active TED. Patients were measured by a single observer, using Hertel exophthalmometry from the time of initial presentation, during the active phase of TED, to the stable phase, 24-months later. Asymmetric proptosis was defined as a >2 mm intra-orbital difference in Hertel measurements., Results: Fifty-one patients were enrolled. Patients presented at a mean time of 1.1 ± 2.9 months following the onset of TED symptoms. Stability of TED was established at 15.7 ± 12.3 months. At initial presentation, 41% of patients demonstrated asymmetric proptosis. Upon reaching the stable phase, asymmetric proptosis persisted in only 22% of patients. A decline in the rate asymmetric proptosis was greatest within the first 3 months of the active phase., Conclusions: Asymmetric proptosis is common in the setting of early active TED and decreases by 50% when the stable phase is reached. Therefore, diagnostic imaging is not routinely required to exclude alternative pathology in the cases of asymmetric TED. Perhaps more importantly, this finding supports the surgical paradigm of stable phase, graded orbital decompression, performed when the ultimate globe positions are achieved to avoid late postoperative asymmetry, resulting from the unanticipated evolution of proptosis when surgery is performed during the active phase of TED.

Periocular haemorrhage without a preceding history of trauma, and in the absence of vascular malformations is rare. While acute proptosis is well described in the setting of orbital pathology, accompanying periocular haemorrhage has been rarely reported. We describe three cases with these concomitant presenting signs in orbital malignancies - metastatic small cell bladder carcinoma, haemangiopericytoma (solitary fibrous tumour) and myeloma. Clinical photographs and radiology are presented with review of the current literature. All cases had an associated rapid onset of severe proptosis and co-existing periocular bruising on the same side. The presence of ecchymosis of the eyelids in addition to proptosis without a history of trauma warrants thorough investigation to rule out underlying potential sight and life threatening illness.