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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. [ABSTRACT FROM AUTHOR]

A 7-year-old male child presented with complaints of lagophthalmos and lid retraction of the right eye since birth. Magnetic resonance imaging (MRI) showed diffuse thickening of right superior rectus and levator-palpebrae complex along with a hypointense, irregular, and ill-defined lesion in the adjoining fat abutting the lacrimal gland. Biopsy from the lesion showed diffuse orbital fibrosis. Another 3-year-old female child presented with complaints of her right eye appearing smaller and inability to move the right eye freely since birth. MRI showed thickening of right superior and medial recti with diffuse retrobulbar hypointense fibrotic strands. The findings were suggestive of orbital fibrosis. Congenital orbital fibrosis is an extremely rare orbital pathology with very few cases described in the literature. The most common clinical features are motility restriction, restrictive strabismus, upper lid retraction, enophthalmos, and proptosis. The diagnosis can be made on imaging but requires biopsy for confirmation. Management is mostly conservative in the form of refractive and amblyopia therapy. [ABSTRACT FROM AUTHOR]

Double hit lymphoma (DHL) and double-expressor lymphoma (DEL) are now considered as aggressive types of diffuse large B cell lymphoma. DHL is characterized by a dual rearrangement of MYC and B cell lymphoma 2 (BCL-2) and/or B cell lymphoma 6 (BCL-6) and DEL by overexpression of MYC and BCL-2. Both DHL and DEL have aggressive presentation and are more common in elderly population. We present a case of 1 ½ years old boy who presented with bilateral proptosis, and diagnosed as non-Hodgkin lymphoma with central nervous system involvement. Immunohistochemistry revealed high expression of MYC and BCL-2. Fluorescence in situ hybridization studies done to rule out DHL showed no translocation of C-MYC, Bcl-2, and Bcl-6 and hence were confirmed as double-expressor high-grade B cell lymphoma. Dual expression of C- MYC, Bcl-2, or Bcl-6 always needs further evaluation to rule out the more aggressive DHL subtypes. [ABSTRACT FROM AUTHOR]

IgG4 related disease (IgG4-RD) is an auto immune fibro-inflammatory condition, characterised by presence of IgG4 positive lymphoplasmacytic infiltrates and extensive fibrosis of the involved organ. It commonly affects pancreas, biliary tract and salivary glands. Sino-orbital involvement is a relatively rare presentation. There is extensive fibrosis of the involved organ. Biopsy is often diagnostic and it shows extensive necrosis and lymphoplasmocytic infiltrates. They show dramatic response to steroid therapy. Here we present three cases of IgG4-RD disease involving orbit and para nasal sinuses who were evaluated and treated in a tertiary care teaching hospital over a period of 2 years. [ABSTRACT FROM AUTHOR]

Dural sinus malformation (DSM) is a rare vascular malformation characterized by the dilatation of a dural venous sinus with or without an anomalous jugular bulb. Its presentation with venous-reflux-related secondary proptosis is anecdotal, with only six such cases reported so far. We report a 17-month-old boy who presented with a progressive proptosis of the right eye secondary to a DSM of the transverse sinus and torcula. Following endovascular embolization of the arterio-venous fistula, complete thrombosis of the venous lake and improvement in proptosis was noted at 6-month follow-up. Prognosis of this rare malformation is variable and dependent on specific angio-architectural features. [ABSTRACT FROM AUTHOR]

PurposeTo explore if orbital fat-to-muscle ratio (FMR) is predictive of whether surgical decompression or teprotumumab leads to greater proptosis reduction in thyroid eye disease (TED).MethodsA single-center retrospective cohort study comparing surgical decompression with teprotumumab according to FMR. All TED patients completing an 8-dose course of teprotumumab between January 2020 and September 2022 and all patients undergoing bony orbital decompression from January 2017 to December 2019 were included. Subjects were excluded if they were

Solitary fibrous tumours (SFTs) are uncommon neoplasms originating primarily in the pleura but occasionally in the orbit. Despite their generally benign nature, they can exhibit aggressive behaviour. This study focuses on a rare case of a giant SFT located in the orbit, emphasizing diagnostic challenges and treatment considerations. A 69-year-old male with a history of progressive eyelid swelling, proptosis, and vision loss underwent a subconjunctival approach for the removal of an orbital SFT. Histopathological analysis, along with immunohistochemistry markers, confirmed the tumour’s identity. Successful tumour resection was achieved through the subconjunctival approach, highlighting the efficacy of this method for orbital SFTs. The histopathological examination revealed typical features of SFT, characterized by spindle cells and unique architectural patterns. Immunohistochemistry further supported the diagnosis. This case underscores the importance of considering orbital SFTs in the differential diagnosis of orbital tumours, particularly when confronted with proptosis and visual disturbances. Surgical excision, guided by radiological imaging and immunohistochemically analysis, remains the primary therapeutic approach. However, due to the potential for aggressive behaviour, further research is needed to optimize management strategies, especially in cases of incomplete resection or atypical behaviour.

Mucoceles are epithelial-lined cavities filled with sterile mucus that develop due to the obstruction of a sinus ostium. Frontal mucocele is one of the most common among mucoceles. Most of them present with ophthalmic manifestations. Here, we present such a case with ocular symptoms. Despite the absence of thyroid-related symptoms or significant medical history, her examination revealed left eye proptosis, limited ocular motility, optic disc edema, and significant visual loss due to optic nerve compression. Further investigations, including orbital computed tomography, confirmed a frontal mucocele extending into the left orbit. Following endoscopic sinus surgery with mucocele excision, the patient experienced significant improvement in vision, resolution of optic disc edema, and proptosis. This case underscores the importance of considering mucoceles in the differential diagnosis of orbital pathologies. It also highlights the efficacy of endoscopic management in achieving favorable outcomes and emphasizes the significance of interdisciplinary collaboration in optimizing patient care.

AbstractEpithelial carcinoma of the nasopharynx originates from the mucosal lining of this region. The etiology of the disease involves a complex interplay of environmental factors, genetic predisposition, and infection with the Epstein Barr virus. In this article, we present a case of nasopharyngeal carcinoma (NPC) that presented with right orbital proptosis and ocular symptoms, without any of the typical signs of nasopharyngeal carcinoma. However, imaging studies revealed subtle findings of NPC. Isolated neuro-ophthalmologic symptoms are not a common manifestation in NPC patients. Proptosis is a rarer finding among ophthalmologic symptoms, usually associated with tumor invasion into the orbit. However, in the presented case, unilateral proptosis was observed without orbital tumor invasion.

Abstract Background Orbital metastasis is a possible complication of small cell lung cancer and a pattern of bilateral invasion of the extraocular muscles has rarely been reported in literature. Case presentation A 46-year-old white male with a past medical history of smoking and stage IV small cell lung carcinoma presented with loss of vision and pain in the left eye. Examination revealed bilateral proptosis and left afferent pupillary defect, and visual acuity was hand motion on the left eye and 4/10 on the right eye. An orbital computed tomography scan showed a compression of the left optic nerve between the extraocular muscles at the apex, and a lateral canthotomy was performed for a new-onset compressive optic neuropathy, with residual visual improvement. There was also significant enlargement of the extraocular muscles in the right orbit. The patient was maintained in palliative treatment with both chemotherapy and local medical and surgical (amniotic membrane cover for exposure keratopathy) ophthalmological treatments until he eventually died 5 months after. Conclusion Bilateral metastasis to the extraocular muscles is a very rare manifestation of small cell lung cancer and the palliative treatment in these cases is challenging.

Thyroid Eye Disease (TED) is a debilitating autoimmune condition often associated with thyroid dysfunction, leading to significant ocular and orbital morbidity. This review explores recent advancements in the management of TED, focusing on both medical and surgical innovations. The introduction of Teprotumumab, the first FDA-approved drug specifically for TED, marks a pivotal development in medical therapy. Teprotumumab targets the insulin-like growth factor-1 receptor (IGF-1R), effectively reducing inflammation and tissue remodeling. Clinical trials demonstrate its efficacy in reducing proptosis and improving quality of life, making it a cornerstone in the treatment of active, moderate-to-severe TED. Surgical management remains critical for patients with chronic TED or those unresponsive to medical therapy. Advancements in orbital decompression surgery, including image-guided and minimally invasive techniques, offer improved outcomes and reduced complications. Innovations in eyelid and strabismus surgery enhance functional and cosmetic results, further improving patient satisfaction. The management of TED necessitates a multidisciplinary approach involving endocrinologists, ophthalmologists, oculoplastic surgeons, radiologists, and other specialists. This collaborative strategy ensures comprehensive care, addressing the diverse aspects of TED from thyroid dysfunction to ocular health and psychological well-being. Future directions in TED treatment include emerging pharmacological therapies targeting different aspects of the disease's pathophysiology and advanced surgical techniques aimed at enhancing precision and safety. This review underscores the importance of a personalized, multidisciplinary approach in managing TED, highlighting current advancements, and exploring potential future innovations to improve patient outcomes and quality of life. [ABSTRACT FROM AUTHOR]

Despite the high prevalence, treatment challenges, and significant impact of eyelid retraction on vision and quality of life among patients with thyroid eye disease, the effects of teprotumumab on eyelid retraction are not fully understood. This study evaluated change in upper eyelid position after teprotumumab. A retrospective study of all patients who completed eight teprotumumab infusions at one institution from January 1 2020 to December 31 2022. Primary outcome was change in upper eyelid position immediately after treatment and at most recent follow-up compared to pre-treatment. Among 234 eyes of 118 patients, average margin reflex distance-1 (MRD1) pre-treatment was 5.25 mm (range 0–10.0, SD 1.75), 4.66 mm (1.0–9.0, SD 1.32) immediately post-treatment (p < 0.001), and 4.50 mm (0–10.0, SD 1.52) at most recent follow-up (mean follow-up duration 10.60 months). In total, 136 (58.12%) eyes of 88 patients had MRD1 reduction immediately post-treatment, averaging 1.49 mm (0.5–5.0 mm, SD 0.97). Every 1-mm increase in pre-treatment MRD1 increased the odds of MRD1 reduction by 15.03% (CI 10.52–19.72, p < 0.001) and increased the reduction amount by 0.48 mm (CI 0.39–0.57, p < 0.001). Of 154 eyes of 78 patients with most recent follow-up, 107 (69.48%) eyes had stable or further improved retraction at most recent follow-up compared to immediately post-treatment. This study found a modest but significant reduction in MRD1 in approximately 60% of eyes, independent of proptosis change, which was sustained by most patients over longer-term follow-up. Higher pre-treatment MRD1 corresponded with greater improvement. These results suggest an overall mild benefit of teprotumumab for upper eyelid retraction. [ABSTRACT FROM AUTHOR]

Infratemporal fossa (ITF) tumors are rare in children and may present with a variety of symptoms. Teratomas are neoplasms derived from the 3 germ layers and approximately 6% to 10% are within the head and neck. Our study discusses one of the first reported cases of teratoma in the ITF in a pediatric patient. A 3-year-old girl presents with 2 years of recurrent monthly left periorbital swelling accompanied by fevers, skin discoloration, and pain. Prior episodes were treated with antibiotics with incomplete resolution. Imaging revealed a cystic lesion centered in the ITF. She was taken for endoscopic endonasal biopsy of the lesion and had no complications. Pathology revealed a mature teratoma composed primarily of pancreatic tissue. Providers should consider masses such as teratoma in the differential for ITF tumors and periorbital edema unresponsive to typical treatment. [ABSTRACT FROM AUTHOR]

Background: Orbital metastasis is a possible complication of small cell lung cancer and a pattern of bilateral invasion of the extraocular muscles has rarely been reported in literature. Case presentation: A 46-year-old white male with a past medical history of smoking and stage IV small cell lung carcinoma presented with loss of vision and pain in the left eye. Examination revealed bilateral proptosis and left afferent pupillary defect, and visual acuity was hand motion on the left eye and 4/10 on the right eye. An orbital computed tomography scan showed a compression of the left optic nerve between the extraocular muscles at the apex, and a lateral canthotomy was performed for a new-onset compressive optic neuropathy, with residual visual improvement. There was also significant enlargement of the extraocular muscles in the right orbit. The patient was maintained in palliative treatment with both chemotherapy and local medical and surgical (amniotic membrane cover for exposure keratopathy) ophthalmological treatments until he eventually died 5 months after. Conclusion: Bilateral metastasis to the extraocular muscles is a very rare manifestation of small cell lung cancer and the palliative treatment in these cases is challenging. [ABSTRACT FROM AUTHOR]

Background Unilateral non-pulsating proptosis can be caused by lesions with intraorbital extensions compressing the globe including sphenoid wing en plaque meningiomas and paranasal sinuses lesions.

Purpose: To report a rare orbital schwannoma arising within inferior rectus muscle in a young woman. Observations: In this case report, we describe a young patient with slowly progressive proptosis for one year. Preoperative imaging was suggestive of a benign tumor arising within inferior rectus muscle, and histopathologic evaluation revealed an orbital schwannoma with cystic degeneration. Conclusion and importance: This case report highlights the importance of including schwannoma in the differential diagnosis of any orbital tumor arising within muscle.

Objective: This study aimed to identify factors affecting proptosis recovery in spheno-orbital meningioma (SOM) surgery and assess functional and oncological outcomes. Methods: Data from 32 consecutive SOM surgery cases (2002–2021) were analyzed. Clinical, radiological, operative, and oncological parameters were examined. Proptosis was assessed using the exophthalmos index (EI) on MRI or CT scans. Statistical analyses were performed to identify predictive factors for proptosis recovery. Results: Proptosis improved in 75% of patients post-surgery (EI decreased from 1.28 ± 0.16 to 1.20 ± 0.13, p = 0.048). Patients with stable or worsened EI had higher body mass index (28.5 ± 7.9 vs. 24.1 ± 4.7, p = 0.18), Simpson grade (IV 75% vs. 65%, p = 0.24), and middle sphenoid wing epicenter involvement (63% vs. 38%, p = 0.12), but no significant factors were associated with unfavorable exophthalmos outcomes. The improvement group had higher en plaque morphology, infratemporal fossa invasion, and radiation treatment for cavernous sinus residual tumor (88% vs. 75%, p = 0.25; 51% vs. 25%, p = 0.42; 41% vs. 25%, p = 0.42, respectively), but without statistical significance. Visual acuity remained stable in 78%, improved in 13%, and worsened in 9% during follow-up. Surgery had a positive impact on preoperative oculomotor nerve dysfunction in 3 of 4 patients (75%). Postoperative oculomotor nerve dysfunction was observed in 25%, of which 75% fully recovered. This occurrence was significantly associated with irradiation of an orbital tumor residue (p = 0.04). New postoperative trigeminal hypoesthesia was observed in 47%, of which 73% recovered. All SOMs were classified as WHO grade 1, and complementary treatments achieved oncological control, requiring gamma-knife radiosurgery in 53% and standard radiotherapy in 6%. Conclusions: Surgery effectively improves proptosis in SOM, though complete resolution is rare. The absence of predictive factors suggests multifactorial causes, including body mass index and tumor resection grade. Postoperative oculomotor nerve dysfunction and trigeminal hypoesthesia are common but often recover. Gamma-knife radiosurgery maintains long-term oncological control for evolving tumor residue. [ABSTRACT FROM AUTHOR]

To explore if orbital fat-to-muscle ratio (FMR) is predictive of whether surgical decompression or teprotumumab leads to greater proptosis reduction in thyroid eye disease (TED). A single-center retrospective cohort study comparing surgical decompression with teprotumumab according to FMR. All TED patients completing an 8-dose course of teprotumumab between January 2020 and September 2022 and all patients undergoing bony orbital decompression from January 2017 to December 2019 were included. Subjects were excluded if they were <18 years, received both surgical decompression and teprotumumab, or lacked orbital imaging. The primary exposure variable was teprotumumab or surgical decompression. The secondary exposure variable was baseline FMR. The primary outcome measure was change in proptosis (mm). Thirty-eight patients, mean age 53.5 years (±11.4), were included in the teprotumumab group and 160 patients, mean age 48 years (±11.1), in the surgical group. Average proptosis reduction after teprotumumab and surgical decompression was 3 mm (±1.44) and 5 mm (±1.75), respectively. The FMR was stratified at the median of 1.80. In subjects with FMR < 1.80, teprotumumab showed equivalent proptosis reduction compared to surgical decompression, −0.33 mm (SE 1.32) p =.802. In subjects with FMR ≥ 1.80, surgical decompression led to significantly more proptosis reduction than teprotumumab, 3.01 mm (SE 0.54), p <.001. Baseline FMR can be used to counsel patients as to proptosis reduction with teprotumumab versus surgery. Subjects with low FMR obtain comparable proptosis reduction with teprotumumab or surgery, whereas high FMR is associated with more significant proptosis reduction following surgery over teprotumumab. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background Unilateral non-pulsating proptosis can be caused by lesions with intraorbital extensions compressing the globe including sphenoid wing en plaque meningiomas and paranasal sinuses lesions. Patients and Methods We operated on 20 patients with unilateral non-pulsating proptosis using fronto-temporo-orbito-zygomatic (FTOZ) approach with orbital reconstruction in six patients. Eighteen patients had sphenoid wing en plaque meningioma, 1 patient had paranasal sinuses fungal infection with intraorbital and intracranial extension, and 1 patient had frontal sinus dermoid with intraorbital extension. Results Proptosis was corrected in 50% of the patients, improved in 25%, and remained stationary in 25%. Vision improved in three patients, remained stationary in three, and deteriorated in one patient. Two patients had temporary oculomotor ophthalmoplegia that resolved within 3 months. Conclusion Although it is an invasive approach, FTOZ gives excellent exposure of the orbit and anterolateral skull base that allows the excision of intracranial lesions with orbital extension. If needed, the orbit could be reconstructed easily due to excellent exposure. [ABSTRACT FROM AUTHOR]

A 13-year-old female patient presented with painless vision loss and proptosis for 18 months. Imaging findings were highly suggestive of a supraorbital aneurysmal bone cyst (ABC) for which she underwent complete surgical excision. Postoperatively, she developed left hemiparesis. Computed tomography angiography (CTA) revealed right complete internal carotid arterial (ICA) thrombosis. This was managed conservatively, and she improved in hemiparesis over the next 3 weeks. Histopathology report revealed osteosarcoma with secondary ABC, for which she was referred for radiotherapy. At 1.5 months follow-up, the patient's left lower limb power improved to 4 + /5. She was walking without support, and her left upper limb power was 4/5. [ABSTRACT FROM AUTHOR]

Sarcoid-like reaction (SLR) has been reported in patients with solid tumor malignancies, lymphomas, and patients receiving immunotherapy. SLR is often incidentally found during positron emission tomography/computed tomography scans as hilar and/or mediastinal lymphadenopathy. SLR has also been found in the lung, spleen, bone marrow, and skin. Biopsy of these lesions shows noncaseating granulomas. When systemic criteria are not met for sarcoidosis, these noncaseating granulomas are termed SLR. We present the first case in the literature of a case of orbital SLR in a patient with concomitant diffuse large B-cell lymphoma and inverted papilloma of the maxillary sinus. This case highlights the importance of including malignancy in the differential for the presence of a noncaseating granuloma in the orbit. [ABSTRACT FROM AUTHOR]

Abstract Background Amyloidosis is a rare condition characterized by the abnormal deposition of amyloid proteins in various tissues and organs. While systemic amyloidosis has been well-documented, amyloid deposition in extraocular muscles is an exceptionally rare occurrence, with only 35 reported cases. This case report sheds light on the importance of considering amyloidosis in the differential diagnosis of patients presenting with proptosis and diplopia, which are often associated with thyroid eye disease. Case presentation A woman in her twenties sought medical attention due to a complaint of diplopia. Her ocular examination revealed almost normal findings except for exotropia and proptosis. Orbital magnetic resonance imaging displayed fusiform enlargement of nearly all eye muscles, a presentation typically observed in thyroid eye disease. However, despite corticosteroid therapy, her symptoms showed no improvement. Given the unusual lack of response to conventional treatment, and inhomogeneous enhancement of the muscle, an extraocular muscle biopsy was conducted. This biopsy yielded a unique finding—amyloid deposition within the muscle tissue. This discovery was particularly intriguing due to the extreme rarity of amyloidosis affecting extraocular muscles, with fewer than three dozen documented cases worldwide. Conclusion This unique case underscores the critical need for a comprehensive approach to diagnosing patients with proptosis and diplopia. While these symptoms are commonly attributed to thyroid eye disease, it is essential to consider alternative diagnoses such as amyloidosis, especially when standard treatments fail to yield results. The discovery of amyloid deposition in the extraocular muscles, although exceedingly rare, emphasizes the significance of a thorough differential diagnosis. In conclusion, this case report highlights the importance of vigilance in clinical practice, encouraging ophthalmologists to explore less common diagnostic possibilities when faced with challenging cases. Further research and clinical investigation are warranted to better understand the mechanisms and potential treatments for amyloidosis affecting the extraocular muscles.

Zellweger syndrome or cerebrohepatorenal syndrome is a rare, multisystem disorder occurring due to defect in metabolic pathway within the peroxisomes. Cirrhosis with portal hypertension is an important presentation of these patients. Given its progressive, multisystem nature, the role of liver transplantation (LT) in Zellweger syndrome remains undefined and controversial. An 11-y-old boy diagnosed with Zellweger syndrome presented to the authors with decompensated cirrhosis along with bilateral proptosis. After a meticulous evaluation, he was offered an ABO incompatible liver transplantation with his mother being the donor. He had an uneventful post operative period. After a follow up of 24 mo, he has normal graft function, normal cognition along with resolution of proptosis. Therefore, in a group of carefully selected patients with Zellweger syndrome, a liver transplantation can be offered successfully with an excellent prognosis. [ABSTRACT FROM AUTHOR]

Background: Amyloidosis is a rare condition characterized by the abnormal deposition of amyloid proteins in various tissues and organs. While systemic amyloidosis has been well-documented, amyloid deposition in extraocular muscles is an exceptionally rare occurrence, with only 35 reported cases. This case report sheds light on the importance of considering amyloidosis in the differential diagnosis of patients presenting with proptosis and diplopia, which are often associated with thyroid eye disease. Case presentation: A woman in her twenties sought medical attention due to a complaint of diplopia. Her ocular examination revealed almost normal findings except for exotropia and proptosis. Orbital magnetic resonance imaging displayed fusiform enlargement of nearly all eye muscles, a presentation typically observed in thyroid eye disease. However, despite corticosteroid therapy, her symptoms showed no improvement. Given the unusual lack of response to conventional treatment, and inhomogeneous enhancement of the muscle, an extraocular muscle biopsy was conducted. This biopsy yielded a unique finding—amyloid deposition within the muscle tissue. This discovery was particularly intriguing due to the extreme rarity of amyloidosis affecting extraocular muscles, with fewer than three dozen documented cases worldwide. Conclusion: This unique case underscores the critical need for a comprehensive approach to diagnosing patients with proptosis and diplopia. While these symptoms are commonly attributed to thyroid eye disease, it is essential to consider alternative diagnoses such as amyloidosis, especially when standard treatments fail to yield results. The discovery of amyloid deposition in the extraocular muscles, although exceedingly rare, emphasizes the significance of a thorough differential diagnosis. In conclusion, this case report highlights the importance of vigilance in clinical practice, encouraging ophthalmologists to explore less common diagnostic possibilities when faced with challenging cases. Further research and clinical investigation are warranted to better understand the mechanisms and potential treatments for amyloidosis affecting the extraocular muscles. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Schwannomas are rare tumors in the orbit, typically originating from various nerves and presenting diagnostic challenges. We present a unique case of a unilateral orbital schwannoma arising from the supraorbital nerve. A 55‐year‐old female presented with a painless, slowly growing mass in the right superior orbit, causing proptosis. Visual acuity remained unimpaired, and clinical examination revealed a well‐defined mass in the superior orbit. A provisional diagnosis of an orbital dermoid or cyst was made, leading to excision biopsy. The histopathological examination confirmed a diagnosis of benign schwannoma. Schwannomas in the orbit, particularly those arising from the supraorbital nerve, are uncommon and often challenging to diagnose. Early surgical intervention is crucial to prevent complications associated with tumor growth. This case underscores the need to consider schwannomas as a differential diagnosis for slow‐growing orbital masses in adults and emphasizes the importance of timely management to prevent vision‐threatening complications. [ABSTRACT FROM AUTHOR]

Thyroid eye disease (TED) is an inflammatory condition involving the periocular and orbital soft tissues, affecting most commonly patients with hyperthyroid disorders. Traditional treatments used for the active phase of the disease range from conservative lubrication for mild symptoms to systemic immunomodulating drugs for moderate-to-severe symptoms. Teprotumumab (Tepezza) is a monoclonal antibody with an inhibitory effect on insulin-like growth factor 1 and is the first Food and Drug Administration (FDA) approved targeted medical therapy for reducing the inflammatory signs and symptoms associated with TED. Two large multicenter, randomized, double-masked, placebo-controlled trials have confirmed the efficacy and safety of teprotumumab in patients with active, moderate-to-severe TED. Recent reports and publications have also demonstrated the efficacy of teprotumumab in a wider range of patients. In this review, we summarize the clinical features and pathophysiology of TED, disease course, and traditional management methods. We further detail the development of teprotumumab, the founding studies that brought it to its FDA approval, adverse events profile, and ongoing as well as future investigations. [ABSTRACT FROM AUTHOR]

Background: Fibro-osseous lesions include a variety of bone lesions with different clinical and histopathological features. Case Description: We report a case of cemento-ossifying lesion involving the left ethmoid and sphenoid sinus in a 17-year-old male patient. Computed tomography showed an expansile hyperdense lesion with sclerosed peripheral mantle epicentered on the left ethmoid and sphenoid sinus and extending into the left nasal cavity. Magnetic resonance imaging was done for preoperative planning. Transnasal endoscopic resection was performed and histopathological examination confirmed the diagnosis of ossifying fibroma (OF). Conclusion: Involvement of the ethmoid sinus with OF is a rare condition; therefore, we examined the literature for similar cases to highlight the possible clinical presentation and management. Endoscopic management is a safe, effective approach with a low rate of complications. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction: IgG4-associated ocular inflammatory pseudotumor is a rare, non-neoplastic inflammatory process with an incidence of 0.28-1.08 per 100,000 people. The diagnosis of this pathology is one of exclusion, having to meet histopathological criteria, enlargement of a specific organ, and serious concentrations of IgG4. Treatment aims to avoid secondary complications. Clinical case: A 54-year-old woman with no previous reported pathologies, reports that for the past 6 months she has itching at the level of the eyeball and the left eyelid, suddenly and without apparent cause, in addition to decreased visual acuity and retro-ocular pain; this is why she asked for the evaluation. The physical examination revealed normal eye movements, left proptosis, pain on palpation, subconjunctival hyperemia, corneal opacity, absence of red reflex, and the left eye being more resistant to pressure in relation to the contralateral eye. The cranio-thoraco-abdominal tomography reported images at the left ocular level that were suggestive of choroidal melanoma without findings of metastatic activity. Enucleation of the left eye was performed. The specimen was sent to pathology which reported diffuse lymphoplasmacytic infiltrate positive for IgG4. Conclusion: Ocular IgG4 disease is a very rare pathology with non-specific symptoms and complex diagnosis. However, a quick and correct approach is essential to avoid complications. [ABSTRACT FROM AUTHOR]

Abstract 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.

Direct or type A CCFs are the direct connection between the cavernous segment of the internal carotid artery and the cavernous sinus. While most direct CCFs are caused by trauma, spontaneous direct CCFs are extremely rare in infants. In this report, we describe a 6-month-old child with bulges in the right eye that had been present since 20 days after birth. On examination, there was a right eye abduction limitation with no deviation associated with proptosis. Bruit was present during auscultation. CEMRI showed an enlarged right cavernous sinus with dilatation of the superior ophthalmic vein, suggesting a carotid-cavernous fistula. The patient was referred to an advanced center where he was advised patch therapy to prevent amblyopia. He was kept under observation by a neurosurgeon until 3 years, after which he was scheduled to undergo transarterial coiling.

AIM: To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma. METHODS: The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital, from 2006 to 2021 were collected and analyzed. The data includes age, gender, affected orbital side, course of disease, status of disease (primary or recurrent), clinical manifestations, preoperative visual acuity, operative treatment, the relations between liposarcoma and surrounding tissue, longest diameter of liposarcoma, histological subtype, immunohistochemical indicators, follow-up treatment and prognosis. RESULTS: The initial symptoms are diverse. Proptosis is the most frequent chief complaint and the others included vision loss, epiphora, diplopia, and eyelid palpable mass. Results of imaging examination [computed tomography (CT) or magnetic resonance imaging (MRI)] showed orbital mass. In terms of treatment, 10 patients received tumor resection, and the mean longest diameter of the tumor was 3.39±1.36 cm. The other 3 patients had optic nerve invaded, so they received orbital exenteration. Pathological examination results confirmed the diagnose of liposarcoma for 13 patients. Six patients displayed as myxoid type, and three patients in each type of dedifferentiated and well-differentiated type. One patient was verified as pleomorphic, which was a rare type of liposarcoma. All of the patients showed Vimentin positive, and most showed CD34 and S-100 positive. Besides, four patients showed smooth muscle actin positive. All thirteen patients were alive. CONCLUSION: Orbital liposarcoma is a rare disease and it has no specific clinical manifestation. The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit. It is recommended to perform pathological examination to achieve early detection and early treatment.

Case Presentation: A 73-year-old male presented to the emergency department complaining of pain in his right eye for four weeks. He denied any trauma, and the pain was accompanied by ptosis, proptosis, swelling, redness, blurred vision, and a frontal headache. On examination, conjunctival arterialization was also appreciated. Magnetic resonance imaging and angiography showed evidence of a carotid cavernous fistula for which the patient underwent successful transvenous coiling and embolization.Discussion: Carotid cavernous fistulas are classified as higher flow, direct fistulas or lower flow, indirect fistulas; the latter is more insidious in onset. Classic symptoms include conjunctival arterialization, proptosis, ptosis, palpebral edema, ocular palsy, vibratory sensation, elevated intraocular pressure without pupillary or visual acuity deficits, and headache. The treatment of choice is transvenous embolization.

An 84-year-old male with previously documented poor medical follow-up presented with progressive painless proptosis of the right eye. Right upper eyelid ptosis, limited motility, proptosis, and inferomedial displacement of the right globe were noted on the exam. Computed tomography (CT) imaging revealed a right retrobulbar extraconal heterogenous mass with ill-defined borders. Biopsy revealed a malignant adenocarcinoma with tumor markers suggestive of a colorectal primary. A rectal mass was identified during a systemic workup. After biopsy, the patient was diagnosed with stage IV metastatic rectal adenocarcinoma. He began palliative radiation therapy shortly following diagnosis.

Carotid cavernous fistula (CCF) is an abnormal vascular shunt from the carotid artery to the cavernous sinus. The characteristic clinical features include chemosis, proptosis, ocular bruit, headache, diplopia, and ophthalmoplegia. The classical radiological features on magnetic resonance imaging (MRI) include proptosis, extraocular muscle enlargement, dilatation of the superior ophthalmic vein, and ipsilateral cavernous sinus enlargement. We report the case of a 66-year-old female who presented with headache, redness of the right eye, and double vision, who had features of CCF on MRI.

Orbital epidermoid cysts are uncommon lesions within the bony orbit with varied symptomatology related to both the eye and the sino-nasal system. They are often slow-growing cystic masses which may cause facial asymmetry and visual loss due to pressure symptoms. Cross-sectional imaging such as computed tomography and magnetic resonance imaging are contributory and useful for assessment of the size and actual extent and should be mandatory before planning any surgical intervention. Open approaches and needle aspiration have been traditionally described; however, the use of the rigid nasal endoscope in the intraorbital compartment is a useful adjunct for exploration of the extent of the lesion and for complete surgical clearance. In this report, a 69 years old female with an old post- traumatic orbital epidermoid cyst which was removed completely using an endoscope via transorbital route was presented with the review of literature.

Abstract Introduction To evaluate the ability of artificial intelligence (AI) software to quantify proptosis for identifying patients who need surgical drainage. Methods We pursued a retrospective study including 56 subjects with a clinical diagnosis of subperiosteal orbital abscess (SPOA) secondary to sinusitis at a tertiary pediatric hospital from 2002 to 2016. AI computer software was developed to perform 3D visualization and quantitative assessment of proptosis from computed tomography (CT) images acquired at the time of hospital admission. The AI software automatically computed linear and volume metrics of proptosis to provide more practice-consistent and informative measures. Two experienced physicians independently measured proptosis using the interzygomatic line method on axial CT images. The AI software and physician proptosis assessments were evaluated for association with eventual treatment procedures as standalone markers and in combination with the standard predictors. Results To treat the SPOA, 31 of 56 (55%) children underwent surgical intervention, including 18 early surgeries (performed within 24 h of admission), and 25 (45%) were managed medically. The physician measurements of proptosis were strongly correlated (Spearman r = 0.89, 95% CI 0.82–0.93) with 95% limits of agreement of ± 1.8 mm. The AI linear measurement was on average 1.2 mm larger (p = 0.007) and only moderately correlated with the average physicians’ measurements (r = 0.53, 95% CI 0.31–0.69). Increased proptosis of both AI volumetric and linear measurements were moderately predictive of surgery (AUCs of 0.79, 95% CI 0.68–0.91, and 0.78, 95% CI 0.65–0.90, respectively) with the average physician measurement being poorly to fairly predictive (AUC of 0.70, 95% CI 0.56–0.84). The AI proptosis measures were also significantly greater in the early as compared to the late surgery groups (p = 0.02, and p = 0.04, respectively). The surgical and medical groups showed a substantial difference in the abscess volume (p

Key Clinical Message Schwannomas are rare tumors in the orbit, typically originating from various nerves and presenting diagnostic challenges. We present a unique case of a unilateral orbital schwannoma arising from the supraorbital nerve. A 55‐year‐old female presented with a painless, slowly growing mass in the right superior orbit, causing proptosis. Visual acuity remained unimpaired, and clinical examination revealed a well‐defined mass in the superior orbit. A provisional diagnosis of an orbital dermoid or cyst was made, leading to excision biopsy. The histopathological examination confirmed a diagnosis of benign schwannoma. Schwannomas in the orbit, particularly those arising from the supraorbital nerve, are uncommon and often challenging to diagnose. Early surgical intervention is crucial to prevent complications associated with tumor growth. This case underscores the need to consider schwannomas as a differential diagnosis for slow‐growing orbital masses in adults and emphasizes the importance of timely management to prevent vision‐threatening complications.

Purpose: We report an unusual case of severe proptosis during phacoemulsification in a 58-year-old female with a history of Crohn's disease, bilateral chronic panuveitis, prior bilateral central retinal vein occlusion, and uncontrolled steroid-associated ocular hypertension requiring bilateral Ahmed glaucoma drainage device (GDD) implantation with pars plana tube placement. Observations: During phacoemulsification of the right eye, the patient developed significant proptosis. Following lid speculum removal and mechanical eyelid manipulation, the proptosis resolved within 20 minutes without requiring a lateral canthotomy. The patient had no permanent visual complications. Conclusions and Importance: The likely pathophysiology of intraoperative proptosis in this case was accumulation of fluid in the retrobulbar space due to a functioning Ahmed tube shunt with the tube placed in the vitreous cavity. To avoid this complication, concurrent cataract surgery may be considered for patients with pars plana tube placement GDD surgery.

Objective: Orbital decompression aims to control ocular proptosis, the most commonly associated sign in Graves’ disease, and enable ocular rehabilitation. While various surgical approaches have been studied separately, no studies have compared them. This study compared the functional results of two surgical approaches for the treatment of ocular proptosis secondary to Graves’ disease, namely orbital decompression achieved by inferomedial versus superolateral wall removal. Methods: This study included 39 patients who underwent orbital decompression due to Graves’ disease. Twenty-four decompressions were performed by removing the inferomedial orbital walls using an endoscopic endonasal approach. Fifteen decompressions were completed by removing the superolateral orbital walls using a frontolateral approach. T-tests were used to compare means, whereas Fisher’s exact test and the chi-square test were used to compare proportions. Results: Patients who underwent inferomedial orbital decompression presented a higher risk of new-onset strabismus/diplopia (16.7%) than those who underwent decompression via the frontolateral approach (0%). Although no cases of preoperative strabismus worsened after superolateral decompression, all cases of preoperative strabismus worsened after inferomedial decompression. Conclusions: Both approaches achieve a reasonable decompression limit. Superolateral orbital decompression tended to offer better outcomes for diplopia and strabismus than inferomedial decompression. Due to the high risk of worsening strabismus and diplopia in patients presenting with these symptoms preoperatively, they should be treated with the transcranial approach whenever possible.

Background: Spheno-orbital meningioma (SOM) is a rare intracranial pathology with intraosseous hypertrophy and intraorbital extension. Method: We described a middle-aged female with SOM who was managed in a micro-neurosurgical manner. The titanium implant was customized and applied to rebuild the orbital wall to prevent postoperative enophthalmus. Conclusion: Despite technical demands, favorable cosmetic, and clinical outcomes without complications can be achieved by meticulous surgical technique following radical resection. Clinical trial registration: NA [ABSTRACT FROM AUTHOR]