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

A 75-year-old male with a history of poorly controlled diabetes, hypertension, coronary artery disease, chronic obstructive pulmonary disease, and obesity presented with severe bilateral periorbital edema with necrosis and purulent discharge. Although hemodynamically stable, laboratory markers indicated systemic toxicity. Imaging showed bilateral periorbital edema extending into the frontal scalp, temporal fossa, and right orbit. Suspected to have necrotizing fasciitis, the patient underwent urgent debridement of bilateral upper and lower eyelids and was found to have postseptal extension of necrosis into the right orbit. During his hospitalization, he was treated with broad-spectrum antimicrobials and underwent a second surgery for exploration and debridement. The patient was lost to follow-up and found to have healed by secondary intention without any surgical reconstruction. Our case demonstrates not only a rare case of necrotizing fasciitis involving all 4 eyelids, but also an exceptional cosmetic and functional result after secondary intention healing., 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.)

Purpose: To review all cases of Erdheim-Chester disease (ECD) with orbital involvement treated at Bascom Palmer Eye Institute in Miami, Florida from 2014 to 2022 and compare presentations, treatment modalities, and outcomes., Methods: A retrospective chart review of all patients diagnosed with ECD who presented to Bascom Palmer Eye Institute from 2014 to 2022 was performed. Data collected included demographics, pretreatment history and ophthalmic examination, pathology report, treatment, subsequent examination, and relevant laboratory results. Histopathology, treatments, and outcomes were reviewed and compared between patients., Results: Four cases were included. Primary treatments included vemurafenib (n = 2), cobimetinib (n = 1), and prednisone (n = 1). All patients demonstrated improvement of ophthalmic symptoms. Vemurafenib was the only medical treatment that was tolerated well and resulted in significant improvement in proptosis despite some reported dry eye; all other medications were discontinued due to intolerable side effects., Conclusions: BRAF inhibitors such as vemurafenib have been used as novel therapy in the treatment of ECD. Vemurafenib demonstrated its utility in reducing proptosis in ECD patients at one ophthalmic institution. Vemurafenib may be a favorable treatment option for BRAF -positive ECD patients presenting with orbital disease., Competing Interests: STW is a consultant for Lassen Therapeutics and Immunovant, an advisor for Horizon Therapeutics, and participates in funded research with Horizon Therapeutics, Immunovant, and Sling Therapeutics. The remaining authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

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.)

Purpose: Juvenile xanthogranuloma (JXG) is a subtype of histiocytosis characterised histologically by foamy non-Langerhan cells with Touton giant cells. It typically manifests as a single self-limiting cutaneous nodule in the pediatric population. Orbital JXG is extremely rare, and its clinical course and management are not well understood or defined. Herein we present 3 cases of orbital JXG and provide a detailed literature review., Methods: Review of 3 cases with orbital JXG and literature review of all published cases., Results: Three presented cases demonstrate the heterogeneous clinical course of orbital JXG. Although centred around the use of steroids, there is neither robust evidence nor consensus on its management. The wider JXG literature is currently concentrated around the classification of JXG with respect to histiocytosis, especially the exclusion of extracutaneous JXG as separate diseases. This separation is based on clinical, histopathological, and molecular findings. It is unclear where orbital JXG best fits in this emerging classification of JXG., Conclusion: Our review of the cases and literature on orbital JXG show that it may manifest with variable clinical course and its molecular pathogenic mechanism may be different to that of the cutaneous JXG., 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: Orbital involvement of invasive fungal sinusitis (IFS) is an ominous prognostic marker that should prompt rapid intervention. Transcutaneous retrobulbar administration of amphotericin B (TRAMB) is an off-label adjunctive treatment that can increase drug penetrance into diseased orbital tissue. To date, there is a lack of consensus regarding the use of TRAMB for treatment of IFS with orbital involvement., Objective: This systematic review aims to synthesize the indications, efficacy, and potential complications of TRAMB., Methods: PubMed, EMBASE, and Web of Science databases were probed for systematic review. Article search was conducted through June 2023 using the keywords "invasive fungal sinusitis," "invasive fungal rhinosinusitis," "rhino-orbital mucormycosis," "rhinosinusitis," "orbital," "retrobulbar," and "amphotericin.", Results: In suitable cases as determined by radiologic and clinical evaluation, TRAMB administration has the potential to improve orbital salvage rates and improve versus stabilize visual acuity. Treatment complications are more likely with deoxycholate than with liposomal amphotericin formulations. The existing literature describing use of TRAMB is limited due to its retrospective nature, but the increase in IFS cases since 2020 due to the COVID pandemic has broadened the literature., Conclusions: TRAMB is an effective adjunctive treatment in IFS with mild-to-moderate orbital involvement when used in combination with standard of care debridement, systemic antifungal therapy, and immunosuppression reversal. Prospective longitudinal studies and multi-institutional randomized trials are necessary to determine the definitive utility of TRAMB.

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

Competing Interests: The authors report no conflicts of interest.

A 54-year-old female noticed a 2-month history of an enlarging left inferomedial orbital rim mass. The patient remembered a pencil injury at approximately 7 years of age. Her complete ophthalmic examination was otherwise unremarkable. She underwent CT orbital imaging, demonstrating a centrally hyperdense lesion along the left inferomedial orbital rim. There was no involvement of the nasolacrimal duct system. The patient underwent an excisional biopsy. The pathology disclosed noncaseating granulomatous inflammation to particulate black material consistent with graphite., Competing Interests: D.M.S. is a consultant and speaker for Amgen. The remaining authors have no conflicts of interest to disclose., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: Topical prostaglandin analogues are commonly used to treat patients with glaucoma, but may cause periocular and periorbital complications known as prostaglandin-associated periorbitopathy syndrome (PAPS)., Methods: A literature review was conducted on PAPS. Given the lack of consensus on grading PAPS, glaucoma specialists from Asia convened to evaluate current PAPS grading systems and propose additional considerations in grading PAPS., Results: Existing grading systems are limited by the lack of specificity in defining grades and consideration for patients' subjective perception of symptoms. Patient-reported symptoms (e.g., via a self-assessment tool) and additional clinical assessments (e.g., exophthalmometry, lid laxity, differences between tonometry results, baseline measurements, and external ocular photographs) would be beneficial for grading PAPS systematically., Conclusions: Effective management of PAPS could be facilitated by a common clinical grading system to consistently and accurately diagnose and characterise symptoms. Further research is required to validate specific recommendations and approaches to stage and monitor PAPS.

Rhino-orbital-cerebral mucormycosis (ROCM) is a rare, invasive, and fatal fungal disease that is often easily misdiagnosed in the early stages due to the lack of specific clinical manifestations and adequate auxiliary examinations. Early diagnosis and timely therapy are essential for successful treatment. In this report, we presented a 46-year-old man with diabetes who experienced gradual vision loss, right ptosis, swelling, and headaches that progressively worsened to death within 4 days after admission. It was finally confirmed as a fungal Rhizopus arrhizus infection by metagenomics next-generation sequencing (mNGS). Our report has proved that mNGS testing should be strongly recommended in highly suspected patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Li, Ge, Xin and Jiang.)

Competing Interests: Competing interests: None declared.

Ocular malakoplakia, a rare inflammatory disorder characterized by Michaelis-Gutmann bodies, is presented in 2 unique cases involving uncommon anatomical sites-the orbit and caruncle. The first case describes a 10-year-old girl with localized subconjunctival swelling near the caruncle, prompting surgical excision, and revealing characteristic malakoplakia features. Despite medical management, surgical intervention provided symptom relief. The second case involves a painless swelling below the lower lid in a 23-year-old female, initially suggestive of lymphoma. Excision biopsy confirmed malakoplakia, emphasizing the potential for clinical misdiagnosis. Histopathological examination showcased Michaelis-Gutmann bodies, von Hansemann cells, and chronic inflammation, confirming the ocular malakoplakia diagnosis. These cases underscore the rarity of ocular malakoplakia, particularly in pediatric patients, and highlight the importance of accurate diagnosis and appropriate management., 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.)

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

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

A 71-year-old Japanese man presented with a 2-month history of diplopia. He had been followed up at another hospital for ocular sarcoidosis for 3 years. On initial consultation, the best-corrected decimal visual acuity was 0.3 on OU. Slit-lamp and funduscopic examinations were unremarkable. The left lacrimal gland was easily palpable. The patient had restricted infraduction in the OD. MRI showed thickened superior ophthalmic veins on both sides and an enlarged left lacrimal gland. Blood tests showed elevated soluble interleukin-2 receptors. Biopsy of the enlarged lacrimal gland showed numerous epithelioid granuloma and lymphocytic infiltrates consistent with sarcoidosis. No other systemic sarcoidal lesions were detected. The patient started to take prednisolone of 30 mg/day, and the dose was tapered by 5 mg every 2 weeks. At 1 month after taking prednisolone, imaging showed no thickening of the superior ophthalmic veins. At 5 months follow-up, the left lacrimal gland was not palpable, limitation of infraduction improved, and diplopia resolved., 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 primary orbital respiratory cyst is a congenital choristoma that presents in the orbit and with different signs and symptoms depending on the location, which might also change the surgical approach. The aim of this report is to describe 2 new cases of primary respiratory epithelial cysts and to review the literature on presentation, management, and risk factors with different surgical approaches and complications. Two cases presenting with gradually increased proptosis had a confirmed diagnosis of a respiratory epithelial cyst. CT and MRI imaging revealed a thin-walled intraconal cystic lesion. Complete surgical removal was not possible and an incisional biopsy of the wall was performed. Rarely, orbital respiratory epithelium cysts are primary lesions. The high risk of breaking the capsule during surgery and the presence of residual epithelial cells within the orbit may cause cyst's recurrence. Indeed, we recommend careful attention during dissection maneuvers and a long-term follow-up., 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.)

Purpose: Within the large umbrella of histiocytosis are a few similar yet heterogenous entities involving the orbit and periocular tissues with or without systemic infiltration, termed adult onset xanthogranuloma or orbital xanthogranuloma. Due to rarity of these conditions, different classifications in use, diverse clinical presentations and still unknown etiology, the aim of this paper was to provide an up-to-date literature review of the actual understanding of histiocytosis and its subgroups involving the orbit and periocular area, diagnostic strategies and therapeutic modalities., Methods: We present a review of literature and small case series comprising four patients diagnosed and treated in the period from 2001 until 2023 in our hospital. Clinical files of 4 patients with adult-onset xanthogranulomatous disease of the orbit and ocular adnexa (AOXGD) were reviewed retrospectively. Clinical, laboratory, radiological, histopathological, and immunohistochemical findings were reexamined., Results: Reviewing medical records of our patients with AOXGD, we found significant overlap between histiocytosis and different immune disorders. A broad workup should be considered in these patients as they can harbour severe immune disfunctions and hematologic disorders. Preferred treatment modality depends on a histopathologic type of AOXGD, clinical presentation and systemic involvement and should be conducted multidisciplinary., Conclusion: The diagnosis is often delayed because of its rarity and diverse clinical findings. Development of molecular genetic tests, detection of BRAF V600E mutation and different types of kinase mutations, mutations in transcriptional regulatory genes as well as tyrosine kinase receptors have shed a new light on the etiopathogenesis and potential targeted treatment of histiocytosis., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: Epidermoid cysts (EC) and dermoid cysts (DC) typically appear as well-circumscribed lesions on CT. This study aimed to clarify the radiologic and histopathologic characteristics of orbital EC and DC and to determine the correlations between them., Methods: The medical records of 69 patients who underwent surgery for orbital DC or EC at Samsung Medical Center between January 2001 and August 2016 were retrospectively reviewed. The size and location of the cysts, rim enhancement, homogeneity of contents, presence of hemorrhagic or calcific components, radiodensity of contents, and extent of bony remodeling were evaluated using CT. Additionally, the cyst lining and contents were examined histopathologically., Results: Among patients with orbital cysts, EC and DC were diagnosed in 10 (14.5%) and 59 (85.5%) patients, respectively. Further, 50.0% of EC and 79.7% of DC were located in the superotemporal quadrant of the orbit. On orbital CT, the average radiodensity of EC and DC was 18.9 ± 56.2 and -67.9 ± 63.3 HU, respectively. The cystic contents were more frequently homogeneous than heterogeneous in both EC and DC; however, the radiodensity of cysts differed significantly, which may be attributed to sebaceous gland activity. Focal bony notching, bone remodeling under pressure, and bony changes from dumbbell-shaped cysts were observed more frequently in DC than in EC., Conclusions: Radiological and histopathological features are correlated in orbital EC and DC. Therefore, orbital EC and DC can be preoperatively differentiated using CT, based on the average radiodensity and bony remodeling., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

We performed a comprehensive systematic review to identify medication-associated orbital inflammation and to characterize its clinico-radiological features. We reviewed English-language articles describing medication-associated orbital inflammation (i.e., orbital myositis, dacryoadenitis and orbital fat) published to June, 2023. Isolated inflammation of the intraocular structures or globe alone (i.e. uveitis, scleritis, optic neuritis and perineuritis) were excluded. In medication-associated orbital inflammation, the extraocular muscles are preferentially affected, occurring in isolation or in combination with other orbital and/or intraocular structures. Clinico-radiological manifestations may be non-specific; however, certain medications may be distinguished according to the presence of systemic prodrome, laterality, associated intraocular inflammation, and predisposition to involve certain orbital structures. Rapid identification, discontinuation of the provoking medication, and systemic corticosteroid therapy (if appropriate) typically achieves a favorable visual prognosis. As new medications become adopted by clinicians, rare adverse effects will be further delineated.Medication-associated orbital inflammation is an important diagnostic consideration in orbital inflammatory disease. A careful medication history and clinical assessment may be revealing, permitting timely discontinuation of the offending agent and initiation of appropriate management., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: To assess the utility of a marginal full thickness blepharotomy (MFTB) for the treatment of orbital compartment syndrome., Methods: An experimental study design employing a cadaver model for orbital compartment syndrome was used to assess the efficacy of an MFTB. Elevated orbital compartment pressures were created in 12 orbits of 6 fresh cadaver heads. Intraocular pressure, as an analog of orbital pressure, was measured before and after inferior and superior MFTBs were performed. Statistical analysis was performed on the collected data to assess the efficacy of the procedure., Results: Both procedures were found to significantly lower the orbital compartment pressure. MFTB of the inferior lateral eyelid decreased orbital compartment pressure by an average of 62.2 mm Hg (95% CI, 56.9-67.5). MFTB of the superior lateral eyelid following MFTB of the inferior lateral eyelid decreased the orbital compartment pressure by an additional average of 10.3 mm Hg (total average reduction of 72.5 mm Hg; 95% CI, 68.1-76.9)., Conclusions: Orbital compartment syndrome is a time-sensitive vision-threatening emergency that requires prompt diagnosis and intervention to prevent irreversible vision loss. The authors describe the MTFB, a simple one-step procedure that when performed correctly results in a significant decrease in orbital compartment pressure, making it a viable option when canthotomy and cantholysis fails or is unable to be performed., 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.)

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

Despite low prevalence of leprosy worldwide, new cases continue to present and require swift evaluation and diagnosis to prevent complications. Here, we describe a case of lepromatous leprosy with Lucio's phenomenon initially presenting with facial and periorbital edema. A 38-year-old Brazilian woman presented to the emergency department with facial swelling and erythema, initially treated as cellulitis. Due to rapid worsening despite broad-spectrum antibiotics, she underwent soft tissue exploration and biopsy due to concern for necrotizing fasciitis. During her course, she also developed retiform purpura of bilateral upper and lower extremities. Periorbital and lower extremity pathological specimens ultimately revealed acid-fast bacilli consistent with Mycobacterium leprae , and the patient improved with multidrug therapy. This case illustrates the diagnostic difficulty of lepromatous leprosy with Lucio's phenomenon, which can initially present with periorbital edema., 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.)

Mucormycosis is a serious fungal infection caused by fungi in the order of Mucorales. Orbital mucormycosis occurs more frequently in rhino-orbital, sino-orbital, and rhino-orbito-cerebral forms of the disease, while isolated orbital mucormycosis is much less common. Herein, we present four cases of immunocompetent children who developed primary cutaneous mucormycosis, which subsequently invaded and progressed to orbital mucormycosis following direct traumatic injury caused by pecking from Acridotheres tristis (Common Myna). Given the low prevalence of orbital mucormycosis in healthy children, an unknown source of infection and delayed diagnosis followed by late therapeutic interventions could result in life-threatening conditions and serious sequelae.

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.

A 4-year-old boy was referred to our tertiary hospital after a penetrating adnexal injury by a large-breed dog to the left orbital area. There was an increase in lacrimation, which was thought to be due to an inflammatory reaction. However, it was discovered that the lacrimation increased in the reverse-Trendelenburg position and with the Valsalva maneuver. Halo sign and beta transferrin test were positive, which led to the diagnosis of cerebrospinal fluid (CSF) fistula, and the patient was operated using a supraorbital craniotomy. A dural tear was visualized and sutured appropriately, then fibrin glue and an autologous galeal graft were applied to the tear. The CSF oculorrhea stopped postoperatively, and the patient was discharged after 10 days of follow-up. The patient had no recurrent CSF leakage at 4-year follow-up. Although CSF oculorrhea is rare and may be difficult to discern from lacrimation, the presence of pneumocephalus and halo sign should suggest fistula repair., 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.)

Purpose: Histiocytosis is one of the most challenging diseases in medical practice. Because of the broad spectrum of clinical manifestations, systemic involvements, unknown etiology, and complex management, different types of histiocytosis are still a big question mark for us. Orbital histiocytosis is characterized by the abnormal proliferation of histiocytes in orbital tissues. It could affect the orbit, eyelid, conjunctiva, and uveal tract. Orbital histiocytosis can cause limited eye movement, proptosis, decreased visual acuity, and epiphora. In this study, we review the novel findings regarding the pathophysiology, diagnosis, and treatment of different types of histiocytosis, focusing on their orbital manifestations., Method: This review was performed based on a search of the PubMed, Scopus, and Embase databases or relevant published papers regarding orbital histiocytosis on October 9th, 2023. No time restriction was proposed, and articles were excluded if they were not referenced in English., Results: 391 articles were screened, most of them being case reports. The pathophysiology of histiocytosis is still unclear. However, different mutations are found to be prevalent in most of the patients. The diagnostic path can be different based on various factors such as age, lesion site, type of histiocytosis, and the stage of the disease. Some modalities, such as corticosteroids and surgery, are used widely for treatment. On the other hand, based on some specific etiological factors for each type, alternative treatments have been proposed., Conclusion: Significant progress has been made in the detection of somatic molecular changes. Many case studies describe various disease patterns influencing the biological perspectives on different types of histiocytosis. It is necessary to continue investigating and clustering data from a broad range of patients with histiocytosis in children and adults to define the best ways to diagnose and treat these patients., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Purpose: The objective is to analyze the radiological diagnosis of orbital lesions and their correlation with the final histopathological findings. We compared the initial reports by extramural radiologists and an in-house radiologist specialized in orbital imaging to evaluate the diagnostic accuracy in the interpretation of orbital imaging., Methods: This was a retrospective chart review of forty patients referred to a Tertiary Eye Care Center in South India over a period of 7 years. These patients already had their imaging done elsewhere. The imaging was re-evaluated by an in-house radiologist. The radiological findings were correlated with the histopathological diagnosis. The diagnostic accuracy between the two radiologists was compared. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value in differentiating malignant from benign lesions were calculated in both groups. The sensitivity and PPV of the radiological diagnosis for neoplastic and inflammatory lesions in both groups were analyzed., Results: The accuracy in differentiating malignant from benign and inflammatory lesions by our in-house radiologist and extramural radiologists was 95% (κ = 0.9 [0.764, 0.997]) and 50% (κ = 0.036 [-0.160, 0.232]), respectively. The sensitivity and PPV of the radiological diagnosis by our in-house radiologist were 93.31% and 100% for benign lesions and 95.24% and 95.24% for malignant lesions. On the contrary, reports from the extramural radiologists showed a sensitivity and PPV of 76.92% and 66.67% for benign lesions and 14.28% and 60% for malignant lesions., Conclusion: A high radiological diagnostic accuracy is possible when analyzed by radiologists experienced in orbital imaging., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Middle East African Journal of Ophthalmology.)

Background: Echinococcosis, commonly known as hydatid disease, is a zoonotic infection resulting from the tapeworm Echinococcus granulosus. The occurrence of hydatid cysts in the orbital region is uncommon, representing less than 1% of all reported hydatid cases. This report details a unique case of an intramuscular hydatid cyst in the orbital region that led to compressive optic neuropathy., Case Presentation: A 22-year-old male from Kabul, Afghanistan presented with a five-month history of progressive proptosis in his left eye, associated with a gradual decrease in vision over the past three weeks. The left eye exhibited upward globe dystopia, ocular motility limitation, mild conjunctival injection, and chemosis. Diagnosis was achieved through imaging and histopathological examination. Treatment involves surgical removal of the cyst and prolonged albendazole therapy. The postoperative course showed significant improvement in the patient's condition and restoration of his vision., Conclusions: Despite its rarity, this case underscores the importance of awareness and knowledge of hydatid disease among physicians, especially those working in endemic areas. It emphasizes the importance of including hydatid disease in the differential diagnosis of orbital masses, particularly in endemic regions., (© 2024. The Author(s).)

This paper presents treatment strategies for orbital necrotizing fasciitis (NF) in a case of a 33-year-old male diagnosed with orbital NF, which developed after dental root canal treatment. Although orbital NF is rare, it is rapidly progressive and can easily lead to the loss of tissue and visual function, sometimes to a life-threatening extent. Prompt and adequate treatment has been a challenge yet remains quite essential. In addition to the conventional approach to NF, such as immediate antibiotic administration and drainage, orbital NF patients like this case were often treated by incorporating additional steps, which include: 1) performing minimally invasive but adequate removal of necrotic tissue through intraoperative use of ultrasound equipment and postoperative use of proteolytic enzyme-containing ointment for chemical debridement; 2) managing intraorbital pressure by lateral cantholysis and orbital floor removal (decompression); and 3) maintaining the aerobic conditions of the wound after surgical drainage via orbital wall removal. Thus far, satisfactory results in patients with extensive NF of the orbit, including the presented case, were achieved with regards to preserving periorbital tissues, vision, and ocular motility through a multidisciplinary approach. These should be considered as optional means of preserving the orbital tissue and visual function., Competing Interests: Declaration of Competing Interest The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Competing Interests: The authors report no conflicts of interest.

Background: VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a hematoinflammatory disease that typically affects adults. It results from a somatic mutation of the E1 ubiquitin conjugating enzyme encoded by the UBA1 gene. VEXAS is frequently accompanied by myelodysplastic syndrome (MDS). The purpose of this study is to describe the ocular and orbital manifestations of VEXAS patients in a case series in our medical centre., Methods: A retrospective chart review was performed for all patients who were diagnosed with VEXAS syndrome in a tertiary medical centre over two years., Results: Eight patients were identified with VEXAS. In six patients, the diagnosis was confirmed by genomic sequencing. Two patients were identified based on their phenotype. All patients were males. The mean age at diagnosis was 78.7 years. In two patients, the ocular manifestation was the presenting symptom for VEXAS. Seven patients (87.5%) had history of MDS. Systemic inflammation manifestations include: skin rash (n = 5), recurrent fevers (n = 2), relapsing polychondritis (n = 2), pleuritis and pleural effusion (n = 2), poly arteritis nodosa- PAN (n = 1) and thrombophlebitis (n = 1). Seven (87%) patients were presented with periorbital oedema. Three patients showed orbital inflammation. Dacryoadenitis was observed in two patients, and extraocular muscle (EOM) myositis was detected in two patients. Four patients demonstrated ocular inflammation such as: episcleritis, scleritis and anterior uveitis., Conclusion: ocular manifestations in VEXAS include orbital inflammation, dacryoadenitis, myositis, uveitis, scleritis, episcleritis and periorbital oedema. We recommend that in old male patients, with history of haematological disorder, presenting with ocular symptom, VEXAS investigation should be taken into consideration., (© 2024. The Author(s).)

Orbital tuberculosis is a manifestation of extra-pulmonary tuberculosis that is challenging to diagnose and treat. Here, we describe the pivotal role of serial imaging in the diagnosis and treatment of orbital tuberculosis. A 28-year-old male presented with recurrent right upper eyelid swelling and a supraduction deficit associated with a firm painless orbital mass, seen on initial computed tomography to be an extensive superomedial lesion producing mass effect. Biopsy revealed a tuberculosis-like granulomatous inflammation, which, coupled with a positive QuantiFERON gold test, led to empiric anti-tuberculin treatment. Serial radiologic imaging following initiation of treatment showed progressive reduction in mass size, supporting the diagnosis and determining the length of treatment. This rare case demonstrates the utility of serial radiologic exams in the diagnosis and treatment of orbital tuberculosis.

Introduction: Schimmelpenning-Feurstein-Mims Syndrome (SFMS) is a rare neurocutaneous disorder. Herein, we describe a novel case and review the phenotypic spectrum and molecular findings of SFMS from an ophthalmology perspective., Methods: Clinical case including presentation, management, pathology, and genetic analysis is described. A literature search on Schimmelpenning-Feuerstein-Mims and its synonyms, Linear nevus sebaceous syndrome, Organoid nevus syndrome, Jadassohn nevus phacomatosis, and Solomon syndrome, was conducted. An updated review and description of published cases with identified genetic mutations are described., Results: A 13-year-old boy with SFMS presented with acute right eye pain and an enlarging orbital mass. Excisional biopsy of the mass revealed an orbital choristoma. Genetic analysis of the orbital tumor confirmed a KRAS c.35 G>A, p.G12D mutation. A literature search revealed 19 cases of SFMS with mutations in the RAS-pathway. KRAS, HRAS, and NRAS mutations were identified in 74%, 21%, and 5% of patients, respectively. Ophthalmic pathology was seen in 83% of patients. Systemic findings varied and involved the skin, central nervous system, and eyes most commonly., Discussion: SFMS, a rare neurocutaneous disorder, results from postzygotic mosaic mutations in the RAS/MAPK pathway. Patients present with various systemic findings and ophthalmic manifestations occur in most cases. This is the first case description of a KRAS mutation identified in an orbital choristoma in SFMS. The disease is described under various names in the literature, and we propose that all syndromic cases with mosaic RAS mutations be reported under the eponym, SFMS.

Mucormycosis is a rare opportunistic infection caused by Mucorales fungi. Cutaneous mucormycosis typically present as chronic indolent infection, whereas rhino-orbital mucormycosis is rapidly progressive disease often invade the adjacent cerebral tissue associated with high mortality. This case represents the atypical clinical history of rhino-orbital-cutaneous mucormycosis. The patient was presented with a right orbital cellulitis associated with an extensive multiple suppurative deep cutaneous infection and worsening headache. The skin lesion was initiated from a localized abscess at the right periorbital area nine months before admission. Suspicion of fungal infection was raised after weeks of non-responsive antibiotics treatment. Aggressive treatment with exoneration of the right eye and surgical debridement was undertaken. Periodic acid Schiff staining from healthy periorbital tissue revealed ribbon-like hyphae with pauciseptate and 90° branching identified as Mucoraceaefamily. The resolution was seen after four weeks of antifungal treatment with Amphotericin B., Competing Interests: The authors declare no competing interest., (Copyright: Ni Putu Ayu Reza Dhiyantari et al.)

Giant cell reparative granuloma has a very low incidence and is thought to be a response to trauma. While there have been only a few reported cases of orbital giant cell reparative granuloma, we recently observed such a case and analyzed 16 previously reported cases of this type. It is important to note that further investigation is necessary to fully understand the relationship between giant cell reparative granuloma and trauma., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Diagnosis and therapy of orbital diseases is an interdisciplinary challenge, in which i.e. otorhinolaryngologists, ophthalmologists, radiologists, radiation therapists, maxillo-facial surgeons, endocrinologists, and pediatricians are involved. This review article describes frequent diseases which both, otolaryngologists and ophthalmologists are concerned with in interdisciplinary settings. In particular the inflammatory diseases of the orbit including orbital complications, autoimmunological diseases of the orbit including Grave´s orbitopathy, and primary and secondary tumors of the orbit are discussed. Beside describing the clinical characteristics and diagnostic steps the article focusses on the interdisciplinary therapy. The review is completed by the presentation of most important surgical approaches to the orbit, their indications and possible complications. The authors tried to highlight the relevant facts despite the shortness of the text., Competing Interests: A. Eckstein erklärt: Vortragshonorare: Sanofi, Amgen,Sling. Mitarbeit an klinischen Studien: Amgen, Immunovant, Novartis.H.-J. Welboborsky war 2021-2022 Präsident der Deutschen Gesellschaft für Hals-Nasen-Ohrenheilkunde, Kopf- und Halschirurgie., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) may affect the eye or orbit, and ophthalmic manifestations of AAV are associated with higher mortality than other inflammatory eye diseases. Perinuclear ANCA (p-ANCA) vasculitis is an uncommon cause of orbital inflammation. A 70-year-old woman with chronic kidney disease presented with a 1-year history of orbital mass and edema around her OD. Fundoscopy revealed 360° optic disc elevation OD. MRI orbits showed an infiltrative, intra- and extraconal lesion extending through the right orbital apex to the cavernous sinus. Labwork and orbital biopsy were consistent with p-ANCA vasculitis, and the patient's ocular symptoms improved after methylprednisolone. Diagnosis of AAV is complicated by a wide diversity of symptoms, and this case highlights an unusual presentation of p-ANCA vasculitis in the orbit. Ophthalmologists have an important role in diagnosing systemic conditions such as AAV by initiating the proper inflammatory workup., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)