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Squamous cell carcinoma of the orbit is uncommon as there is no squamous epithelium in the orbit. Thus, mechanistically squamous cell carcinoma of the orbit most commonly arises from a cutaneous lesion. Although orbital epidermoid cysts are thought to have very low malignant potential, these lesions possess squamous epithelium and theoretically can undergo malignant transformation. Here, the authors present the case of a 63-year-old woman who presented with a 3-month history of diplopia and forehead tenderness with an orbital extraconal lesion on MRI consistent with a ruptured epidermoid cyst. Six months following resection, she suddenly experienced new-onset left upper eyelid ptosis, recurrent diplopia, and left orbital pain. MRI revealed a recurrence of the left orbital mass. Left anterior orbitotomy and biopsy revealed cystic squamous cell carcinoma. This case appears to demonstrate a very rare malignant transformation of an epidermoid cyst to cystic squamous cell carcinoma., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Nerve sheath myxomas are extremely rare myxoid peripheral nerve sheath tumors with a predilection for the distal extremities, particularly common in the fingers and knees. Here, the authors report a 60-year-old male patient with a subconjunctival epibulbar nerve sheath myxoma, which was clinically diagnosed as an orbital fat prolapse. The lesion was successfully debulked without clinical recurrence over more than 3 years. To the authors' knowledge, this is the first case with subconjunctival presentation and fourth orbital reported case., Competing Interests: The 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.)

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.)

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

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.)

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: 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.)

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.)

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.)

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.)

IgG4-related disease is an uncommon multisystem inflammatory disease characterized by tissue infiltration by IgG4 plasma cells, extensive fibrosis, and sclerosing inflammation. Diffuse orbital involvement extending to the orbital apex and cavernous sinus has been reported, but a solitary well-circumscribed lesion at the apex has not been previously reported. Herein, we report a unique case of IgG4-related orbital disease presenting as a solitary well-circumscribed orbital apex lesion causing subacute visual decline in a 45-year-old male. MRI demonstrated bilateral lacrimal gland and infraorbital nerve enlargement and an 18 × 7 mm left orbital apex lesion demonstrating homogeneous contrast enhancement and isointense signal on T2. He was initially treated with tapering systemic corticosteroid therapy with improvement in his vision; however, he experienced visual deterioration 3 months later with recurrent inflammation. He subsequently commenced high-dose systemic corticosteroid therapy and rituximab infusions with improvement of left visual acuity to 6/7.5 + 2., Competing Interests: The authors have no financial disclosures or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

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

Purpose: The Isabel differential diagnosis generator is one of the most widely known electronic diagnosis decision support tools. The authors prospectively evaluated the utility of Isabel for orbital disease differential diagnosis., Methods: The terms "proptosis," "lid retraction," "orbit inflammation," "orbit tumour," "orbit tumor, infiltrative" and "orbital tumor, well-circumscribed" were separately input into Isabel and the results were tabulated. Then the clinical details (patient age, gender, signs, symptoms, and imaging findings) of 25 orbital cases from a textbook of orbital surgery were entered into Isabel. The top 10 differential diagnoses generated by Isabel were compared with the correct diagnosis., Results: Isabel identified hyperthyroidism and Graves ophthalmopathy as the leading causes of lid retraction, but many common causes of proptosis and orbital tumors were not correctly elucidated. Of the textbook cases, Isabel correctly identified 4/25 (16%) of orbital cases as one of its top 10 differential diagnoses, and the median rank of the correct diagnosis was 6/10. Thirty-two percent of the output diagnoses were unlikely to cause orbital disease., Conclusion: Isabel is currently of limited value in the mainstream orbital differential diagnosis. The incorporation of anatomic localizations and imaging findings may help increase the accuracy of orbital diagnosis., Competing Interests: The authors have no financial or conflicts of interest to disclose, (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

The authors report a rare case of orbital sarcoidosis with caseating granulomatous inflammation. A 55-year-old man presented with a 2-month history of progressively worsening diplopia and proptosis of the OS. Orbital CT demonstrated a diffuse orbital mass. Diagnostic anterior orbitotomy demonstrated caseating granulomas. Infectious testing, including special stains, cultures, and polymerase chain reaction testing, were negative for infectious causes. Chest CT demonstrated the presence of hilar lymphadenopathy with bronchoscopic biopsy showing noncaseating granulomas, supporting a diagnosis of sarcoidosis. The patient achieved clinical and symptomatic improvement at 8-month follow-up on methotrexate. While sarcoidosis is typically characterized by non-necrotizing granulomatous inflammation, sarcoid granulomas with necrosis have been previously described in pulmonary histopathology. This case emphasizes the importance of a comprehensive systemic workup, keeping systemic sarcoidosis on the differential, for necrotizing granulomatous inflammation of the orbit., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To explore the impact of season on the incidence of presentation to emergency departments with sinusitis-related orbital cellulitis in the United States., Methods: The National Emergency Department Sample was queried to identify cases of patients with sinusitis-related orbital cellulitis. Patient's age, location, and the month of presentation were recorded. Statistical correlations were analyzed via a dedicated software package., Results: A total of 439 patients with sinusitis-related orbital cellulitis were identified. The overall incidence was higher during the winter months ( p < 0.05); while children were more likely to develop this disease during the winter ( p < 0.05), season was not statistically correlated with its incidence among adults ( p = 0.16). The incidence of orbital cellulitis was higher during the winter in the midwest and south regions of the United States ( p < 0.05 for each region), although this correlation did not apply in the northeast and west ( p = 0.60 and 0.99, respectively)., Conclusions: While sinusitis incidence increases during the winter, the relationship between season and orbital cellulitis is complex and varies by age and geographic location. These findings may help to facilitate screening protocols for this disease and to define staffing issues for emergent ophthalmic care., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Microphtalmos with orbital cyst is a rare congenital abnormality of the eye and orbit that is caused by incomplete closure of the embryonic fissure. The cysts project through in a coloboma of the affected eye. It may be sporadic or genetic. Herein, the authors present a 32-year-old mother with unilateral and her 4-month-old daughter with bilateral microphtalmos and accompanying orbital cyst., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To describe artifacts on orbital MRI, which led to an incorrect radiology report., Methods: Retrospective chart review of patients identified from the orbital databases at the Royal Adelaide Hospital and University of Wisconsin Hospital. Patients who had artifacts on orbital MRI that led to an incorrect radiology report were included. Records were evaluated for age at imaging, gender, MRI sequence, laterality, and location of artifact, radiological characteristics and misdiagnosis, and cause of artifact., Results: Data were collected from 7 patients (3 male) who had a median age of 61 years at the time of imaging. Five artifacts resulted from fat-suppression failure with 4 of these cases misdiagnosed as inflammatory changes and 1 misdiagnosed as neoplastic infiltration. The OD was involved in 4 cases. Six cases were in the inferior orbit region., Conclusions: Fat-suppression failure artifacts may arise in the inferior orbit region and can be mistaken for inflammatory or neoplastic orbital disease. This may prompt additional investigations such as orbital biopsy. Clinicians should be aware of artifacts which can affect orbital MRI and lead to potential misdiagnosis., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To characterize clinical and radiographic features, management, and outcomes of patients with orbital involvement of multiple myeloma (MM)., Methods: A retrospective chart review identified patients with MM and orbital involvement confirmed by histopathology at a single institution between 1995 and 2021. A comprehensive literature review was performed via PubMed to identify all previously reported cases of orbital MM., Results: Retrospective review identified 7 patients (43% male, mean age 68.7 years). Presenting symptoms included proptosis and diplopia. Orbital lesions were primarily located laterally (42.8%) with associated extraocular muscle (57.1%) or lacrimal gland (42.9%) involvement. Five patients (71.4%) had a previous diagnosis of systemic MM. Six patients received chemoradiation (85.7%). All patients had improvement of orbital disease with 2 patients deceased due to disease at follow-up (mean 8.9 months). Literature review identified 111 cases (46.8% male, mean age 58.6 years). 48.6% presented with orbital disease as the first manifestation of systemic MM. Lesions were most commonly located superolaterally (20.2%) with extraocular muscle infiltration (25.2%), lacrimal gland involvement (7.2%), and orbital bony destruction (39.6%). Treatments included chemoradiation, chemotherapy, or radiation alone. Approximately half (51.4%) of patients experienced improvement in orbital disease following treatment, and 48.6% were deceased at follow-up (mean 20.1 months)., Conclusions: This study provides a new retrospective study and updated comprehensive literature review regarding orbital MM. Given its poor prognosis, characterization of orbital MM is essential for early diagnosis. Orbital MM is often unilateral, located superolaterally, and may represent the first manifestation of systemic disease. Treatment includes chemotherapy and radiation, which may improve orbital disease; however, the overall prognosis remains poor., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

The authors present a case of a non-traumatic, spontaneous subperiosteal orbital hematoma in a woman with a history of chronic pansinusitis and absence of midline nasal cavity structures due to chronic inhalational cocaine use. The patient underwent left orbitotomy and drainage of the lesion, showing mostly blood with a small amount of purulence that grew methicillin-resistant Staphylococcus aureus when cultured. The patient received 4 weeks of intravenous antibiotics in addition to functional endoscopic sinus surgery. At 1 month after surgery, her vision had returned to baseline, and proptosis was resolved. Fewer than 20 cases of subperiosteal orbital hematomas associated with chronic sinusitis have been reported. To the authors' knowledge, this is the first reported case of a subperiosteal orbital hematoma associated with cocaine-induced midline destructive lesions. Patient consent to obtain photographs was obtained and archived. All collection and evaluation of patient health information were compliant with the Health Insurance Portability and Accountability Act, and this report adheres to the Declaration of Helsinki., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

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

Purpose: Following COVID-19 infection a rising count of rhino-orbito-cerebral mucormycosis is observed, requiring orbital exenteration, a disabling lifetime affecting surgery. One of the potential interventions for globe salvage in these patients is retrobulbar injections of amphotericin B. This study was conducted to review protocols, outcomes, and side effects of retrobulbar injection of amphotericin B in patients with COVID-19 associated rhino-orbito-cerebral mucormycosis (CAM)., Methods: The PubMed, Scopus, Web of Science, and Embase databases were searched using a comprehensive string of relevant keywords. All English studies with the confirmed diagnosis of CAM infection were included. We excluded all studies in which retrobulbar injection of amphotericin B was not implemented in any of the patients or there was a lack of clarified and detailed data about this procedure among participants., Results: A total of 647 cases had a history of retrobulbar injection(s) of amphotericin B in 13 reviewed studies with 3,132 subjects of CAM. The most common protocol was the retrobulbar injection of 1 ml of 3.5 mg/ml liposomal amphotericin B for 3 doses daily or on alternate days. We discerned that the globe salvage rate was 95.0% in eyes with a history of retrobulbar injection(s). The total rate of orbital exenteration was 14.9%, regardless of the history of retrobulbar injection of the drug. Other outcomes of this intervention were vision salvage and reduced major ophthalmic complaints, including pain, swelling, chemosis, ptosis, and ophthalmoplegia. The side effects of this intervention were not serious, and most of them were transient. They included swelling at the injection site, restriction of ocular motilities, exacerbation of orbital inflammation, and even intensification of visual impairment in a few cases., Conclusions: Retrobulbar injection of amphotericin B should be considered a nearly safe and protective intervention against orbital exenteration in patients with CAM. It may also be effective in saving vision. Since the effectiveness of orbital exenteration in the survival of patients is not ascertained, retrobulbar injections can be considered an alternative intervention., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

A 78-year-old man presenting for revision ptosis surgery was found to have an asymptomatic left inferomedial orbital mass visible below the left lower eyelid on external inspection, and subconjunctivally on examination. This was subsequently diagnosed as an isolated elastoma. A mass in a similar location was excised 60 years previously. His other ophthalmological history included stable diplopia corrected with prism, left-sided ectropion, bilateral sequential phacoemulsification, and past bilateral ptosis which has been persistent on the left side despite surgical repair and revision. His examination revealed left hypertropia but was otherwise largely unremarkable. However, imaging demonstrated the soft tissue lesion abutting the left globe. An anterior orbitotomy was performed, and the lesion was biopsied and specimens sent for histopathological examination and immunohistochemistry. This is the first case of an elastoma of the orbit reported in the literature to the best of the authors' knowledge., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: Treatment for orbital venolymphatic malformations (VLMs) commonly includes 3 major options: sclerotherapy, surgery, and embolization followed by surgical excision. Each has certain advantages, although it is not clear whether all are effective. The authors characterize the clinical course for a series of patients with distensible orbital venous-dominant VLM treated with sclerotherapy and/or embolization with excision., Methods: In this cross-sectional cohort study, patients affected by distensible orbital venous-dominant VLM presenting to the orbital and ophthalmic plastic surgery service from 2014 to 2020 were identified. Patients were included if they presented with a moderate-flow, distensible venous-dominant malformation associated with Valsalva-related symptoms (e.g., pain, proptosis, and diplopia)., Results: Six cases were treated with sclerotherapy. Four underwent multiple treatments, with a mean ± SD of 3.5 ± 2.3 (range 1-7). All patients in this group failed to improve or experienced recurrence of symptoms after sclerotherapy. Twelve cases were treated with embolization and excision. Resolution of symptoms in all 12 cases was noted and maintained for a mean of 3.4 ± 2.1 years. There have been no cases of recurrence. Patients treated with sclerotherapy were more likely to experience recurrence of symptoms compared to those treated with embolization and excision (p < 0.001)., Conclusions: Treatment of distensible venous-dominant moderate-flow orbital VLM with sclerotherapy may provide temporary improvement in some cases. However, in the medium to long term, recurrence was universal in this series. Embolization with excision appears to provide more definitive management, avoiding recurrence in all cases for a mean follow-up of 3 years., Competing Interests: The authors have no financial or conflicts of interest to disclose. The authors alone are responsible for the content and writing of the paper., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

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

Purpose: To evaluate the clinical presentation, anatomical location, and histological features of congenital conjunctival cysts of the orbit. The location and the histological features of inflammation in these patients were compared with those for 293 orbital dermoid cysts., Patients and Methods: Retrospective review of the clinical details, imaging, and histopathology for patients who had excision of conjunctival cysts from their orbit between 1992 and 2020; patients with a history of trauma or surgery were omitted., Results: Twelve patients (7 male; 58%) with congenital conjunctival cysts were identified, the patients presenting at an average age of 16 years (median 26; range 1-61) with a symptoms for a mean duration of 20 months (median 24; range 6-36). The commonest symptoms were peribulbar lump (6/12 patients; 50%), and eyelid swelling and blepharoptosis (6/12 patients; 50%). An orbitaxl mass was palpable in 10 patients (83%), 3 patients (25%) had mild proptosis (1-3 mm), and the cysts were most commonly located superiorly (6/12 patients; 50%) or superonasally (3/12; 25%) in the anterior half of the orbit. Imaging was performed in 7 cases, this showing an intimate relation to the common sheath of the superior rectus/levator complex in 3 patients (25%) and to the trochlea in 1 (8%). All cysts were excised completely, and no patient had postoperative complications or recurrence. Chronic mild and nonspecific inflammation was evident within the cyst wall in 7 cases (54%), but-unlike 55% of the 293 dermoid cysts-none showed granuloma formation., Conclusion: Congenital conjunctival cysts are rare and usually present with a palpable mass in the upper eyelid sulcus. A significant proportion of these cysts have an intimate relationship with the trochlea, or the superior rectus, levator palpebrae or superior oblique muscles and, to minimize the risk of postoperative diplopia or ptosis, particular care must be exercised during surgery., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Aim: To describe the characteristics of patients presenting with congenital orbital cysts containing epithelia of both cutaneous phenotype-that is, epidermis with or without keratin, and at least one dermal structure (sweat gland or pilosebaceous unit)-and conjunctival phenotype (with goblet cells); these cysts with mixed lining are termed "dermo-conjunctival" cysts., Patients and Methods: Review of clinical records for patients having dermo-conjunctival cysts that were treated between 1997 and 2017; patients with cysts of solely conjunctival or solely cutaneous origin were omitted. Data recorded included gender, age at presentation, laterality, orbital location, ophthalmic features, surgical and radiological data, and light microscopic findings., Results: Of 241 patients with congenital orbital cysts, 22 (9%) contained both cutaneous and conjunctival epithelium; unlike the relatively common congenital cysts lined with solely cutaneous epithelia, these dermo-conjunctival cysts typically occupied the superonasal or nasal quadrants of the orbit (p < 0.000001). Fifteen (68%) of the 22 patients were male, and the group presented at a median age of 22 years (range 8-51 years), with symptoms for a median duration of 5 years (range 1 month-33 years). Fourteen (64%) had noted an orbital mass, 3 (14%) had inflammatory pain, and 1/22 (4%) had reduced acuity. Globe displacement was axial in 7 patients (32%) and nonaxial in 13 (59%), and ocular motility was restricted in 4/22 (18%). Of 17 patients with imaging, 9 (53%) had bone expansion, and the cyst was intimately related to the trochlea in 10 (59%). By definition, all cysts contained conjunctival epithelium with goblet cells: hair shafts were present in 13/22 (59%) specimens, sebaceous units in 18 (82%), sweat glands in 6 (27%), and keratinized epithelium was present in 8 (36%). Fourteen (63%) of cysts had mild chronic inflammation within the cyst wall, and granulomas were present in 8 (36%)., Conclusion: Congenital dermo-conjunctival orbital cysts are rare and favor a medial location-this suggesting an origin from sequestered caruncular tissues, the only postseptal source of both dermal and conjunctival structures. Unlike pure cutaneous cysts that typically present as superficial masses in childhood, dermo-conjunctival cysts are often postseptal and present in adults., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To determine whether the age-adjusted Charlson comorbidity index (age-CCI) in sino-orbital fungal disease patients correlates with disease-specific mortality., Methods: Hospital billing systems at 2 academic institutions were queried for patients with ICD-9, ICD-10, and CPT codes used in fungal disease who also had orbital disease and significant visual loss. Thirty-two patients at Institution A and 18 patients at Institution B met the inclusion criteria of microbiologic or pathologic confirmation of fungal infection and completion of inpatient ophthalmology evaluation. Patients without radiographic abnormality in the sinus or orbit were excluded. Demographic, diagnostic, treatment, and outcome variables were recorded. Our primary outcome was death due to fungal disease., Results: Of the 50 medical records examined, 44 patients met the criteria for fungal-related death outcome on multivariate analysis. The regression coefficient for age-CCI and fungal-related mortality was 0.242 (95% CI, 0.012-0.779) with a p value of 0.038., Conclusions: Age-CCI is significantly associated with fungal-related mortality. This relationship remains significant when controlling for 5 covariates of fungal organism phylum, presence or absence of CNS disease, exenteration, local treatment use, and presence or absence of an immunosuppressive diagnosis. Age-CCI shows promise as a clinical and research tool in the evaluation of invasive fungal disease involving the orbit., (Copyright © 2021 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.

Orbital lymphatic malformations are benign, slowly progressive vascular malformations. Management of these malformations is challenging due to their infiltrative and diffuse nature. The authors present a case with orbital apex lymphatic malformation treated with transnasal endoscopic sclerotherapy., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To describe the demographics, clinical presentation, treatment, and outcomes of a rare cohort with simultaneous orbital and intracranial abscesses., Methods: A historical cohort study of 17 patients with simultaneous orbital and intracranial abscesses between 2010 and 2018 was performed. The demographics, location of abscesses, treatment, and outcomes of these patients were analyzed., Results: The mean age was 26.9 years (range 5-83 years). Fourteen patients (82%) were male. In this cohort, the most common orbital abscess location was the superior orbit, involved in 14 patients (82%). The most common site of intracranial abscess was the frontal lobe, involved in 16 patients (94%). Concurrent sinus disease was present in 16 patients (94%). Surgical evacuation was the standard of treatment, with 94% of patients undergoing at least one surgical procedure. Streptococcus species were the most common, isolated from 6 sinus cultures (43%), 3 orbitotomy cultures (21%), and 4 craniectomy cultures (36%). Staphylococcus species were also common. Most patients (94%) had stable or improved mental status and visual function at the conclusion of their treatment., Conclusions: Simultaneous orbital and intracranial abscesses are rare. Local invasion from the orbit into the intracranial space may occur from direct spread, thus superior orbital abscesses pose the greatest risk for intracranial spread. Additional factors such as infection with Streptococcus and Staphylococcus species as well as male sex appear to be risk factors for intracranial spread. For those who develop intracranial abscesses, young age and absence of seizures or altered mental status at presentation may be associated with favorable outcomes., Competing Interests: Suzanne W. van Landingham and Mark J. Lucarelli’s roles were supported by an unrestricted grant from Research to Prevent Blindness, Inc. New York, New York, to the Department of Ophthalmology and Visual Sciences, University of Wisconsin-Madison. Suzanne W. van Landingham’s role was supported by the Heed Ophthalmic Foundation. Other authors have no conflicts of interest or sources of funding., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

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

Intramuscular choristomatous cystic lesions within the orbit are rare but may require removal due to their volume effects. The authors describe 2 such cases of intramuscular cysts in adult patients. Both patients presented with symptoms due to local mass effects with swelling, proptosis and diplopia. Imaging confirmed cystic intramuscular masses. Surgical removal involved intraoperative decompression of the cyst to aid dissection with excision of some muscle fibers. Histology confirmed a keratinized stratified squamous lined dermoid cyst within lateral rectus for case 1 and stratified squamous non-keratinized conjunctival epithelium (simple primary conjunctival cyst) within inferior oblique for case 2., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2020 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: To describe the occurrence of multiple trigeminal nerves (TGNs) enlargement in patients with orbital IgG4-related disease., Methods: Retrospective review of MRI findings and medical records of 6 patients (10 orbits) with orbital IgG4-related disease and enlargement of more than 1 TGN. Orbital biopsies were performed in all cases revealing the typical lymphoplasmacytic infiltrate with significant plasma cell positivity for IgG4 (IgG4+/IgG ratio ≥ 40%). Three experienced neuroradiologists reviewed the MRI sequences using a digital imaging viewer system (Horos, https://horosproject.org/)., Results: Bilateral involvement of at least 2 TGNs divisions was detected in all 6 patients. Enlargement of both V1 and V2 nerves was diagnosed in 5 patients, and in 3 cases, all TGN divisions were involved. V2 nerves were the most affected. In this division, all 12 infraorbital nerves were enlarged, followed by lesser palatines (10/83.3%), superior alveolar (10/83.3%), and zygomatic (6/50%). V1 and V3 nerves were less affected albeit 9 (75%) frontal branches (V1), and 50% of the inferior alveolar (V3) nerves were also enlarged., Conclusions: Widespread involvement of the TGN is an important feature of IgG4-related disease., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2020 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Competing Interests: The author has no financial or conflicts of interest to disclose.

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

Respiratory epithelial orbital cysts (REOC) are rare benign lesions that may be either congenital or acquired. Congenital REOC develop from embryological epithelial rests and are known as choristomas, whereas acquired forms develop from the sequestration of respiratory epithelium from adjacent paranasal sinuses following trauma and/or fracture repair procedures. The final diagnosis can be difficult because congenital and acquired REOC and mucoceles share the same features histologically. The involvement of the paranasal sinuses, which is the landmark of mucoceles, does not occur with REOC. Moreover, the intracranial extension, which is known for mucoceles, has never been reported for REOC. Respiratory epithelial orbital cysts warrant prompt radical surgical resection given that these lesions may lead to a progressive intracranial progression and/or recurrent infection with a potential for meningitis or cerebral abscess. The authors report a unique case of a patient with an intraorbital posttraumatic respiratory epithelial orbital cyst associated with intracranial involvement., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2020 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: Subperiosteal orbital lesions are most commonly abscesses secondary to sinusitis but, in rare cases, may represent other processes. Here, the authors compare the clinical and radiographic presentation of subperiosteal abscesses and alternate subperiosteal processes ("masqueraders") in an effort to establish distinguishing preoperative diagnostic criteria., Methods: A retrospective chart review of cases of subperiosteal orbital lesions that underwent surgical intervention over a 3-year period was performed. The medical records of 6 cases of subperiosteal masqueraders and 6 cases of abscesses were reviewed for the clinical course, imaging (including radiographic density of lesions), and pathology. Clinical and radiographic features of the 2 groups were compared., Results: All cases presented with orbital signs on exam. Fever and leukocytosis were absent in the masquerader group and present in 3 patients from the abscess group. Common radiographic findings in both groups included a rim-enhancing convex mass along the orbital wall and adjacent sinus opacification, often with bony dehiscence. Of the masqueraders, the final diagnosis was hematoma in 3 cases, mucocele in 1, and malignancy in 2. The difference between the mean radiodensity of the subperiosteal abscesses, 38 ± 5 Hounsfield units (95% CI, 34-42), as compared with the average radiodensity of the masqueraders, 71 ± 5 Hounsfield units (95% CI, 67-75), was significant (p = 0.042). Comparing radiodensity of the orbital lesion to adjacent sinus lesions and metastatic lesions elsewhere was also informative in establishing the diagnosis., Conclusions: Radiographic features, particularly radiodensity, may help distinguish subperiosteal abscesses from other lesions and aid in preoperative diagnosis and management.

A 49-year-old otherwise healthy male presented with an odontogenic abscess and mild left facial swelling. CT imaging revealed gaseous hypodensities within the inferior orbital fissure and pterygopalatine fossa in addition to infection of the left masseter and temporalis muscle. Despite dental drainage, this rapidly progressed to orbital cellulitis with temporalis muscles abscess leading to compartment syndrome and globe tenting. He had an excellent outcome after canthotomy and cantholysis, urgent endoscopic and transconjunctival orbital decompression, temporalis muscle abscess drainage, and intravenous antibiotics. This case describes the use of bony orbital decompression for orbital compartment syndrome and globe tenting from odontogenic orbital cellulitis. In addition, this case radiographically demonstrates a transinferior orbital fissure passageway of an odontogenic abscess in the orbit.