Raccoon Eyes Presentation in a Child Diagnosed With Acute Lymphoblastic Leukemia: A Case Report.

Raccoon's eyes (periorbital ecchymosis) may present as the first sign in patients with skull base/base/facial fractures and tumors. In childhood, orbital metastases of neuroblastoma should be considered in the absence of trauma history. Herein, we report a 3-year-old girl diagnosed with acute lymphoblastic leukemia who presented with periorbital ecchymosis. To the best of our knowledge, this is the first pediatric patient with acute lymphoblastic leukemia in the literature who presented with raccoon eyes.

Pediatric orbital subperiosteal abscess outbreak in Iran: characteristics and causes.

PURPOSE: To evaluate demographics, characteristics, and management of pediatric patients with subperiosteal abscesses (SPA) secondary to orbital cellulitis and discuss the etiology of a dramatic rise in SPA. METHODS: Data were gathered by retrospective chart review of patients admitted to a tertiary referral eye hospital (Farabi Eye Hospital) diagnosed with orbital cellulitis with subperiosteal abscess from October 2022 to March 2023 (six months). Data on demographic information, clinical examination, radiographic evidence of sinusitis, orbital cellulitis, SPA, surgical and non-surgical management taken, isolated bacteria, and duration of hospital stay were gathered. RESULTS: 24 patients were admitted during these six months, with a diagnosis of orbital SPA secondary to paranasal sinusitis, confirmed by an orbital Computed Tomography (CT) scan. The age range was 11 months to 16 years. 75% of patients were male. All patients had a history of flu-like illness before developing orbital cellulitis. All patients had concurrent sinusitis, and 18 underwent initial surgical abscess drainage. The ethmoid sinus was the most involved, and most patients had a medially located SPA. Abscess volume ranged from 0.78 to 7.81 cm3 (mean: 3.52 cm3). One patient had concurrent central retinal artery occlusion due to orbital cellulitis. CONCLUSIONS: In this study, we report a dramatic increase in the incidence of SPA referred to our hospital. Larger abscess volumes and an increased number of cases that needed initial surgical drainage are also of note. An influenza outbreak in the autumn and winter, undiagnosed Corona Virus Disease 2019 (COVID-19) infection, increased antimicrobial resistance due to excessive off-label use of antibiotics during the COVID-19 pandemic, and more virulent bacterial infections are the most probable hypotheses to justify this observation.

Orbital intramuscular hydatid cyst causing compressive optic neuropathy: a case report and literature review.

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.

Clinical Evaluation and Surgical Management of Ocular Malakoplakia of the Caruncle and Periorbital Region: A Report of Two Case and Literature Review.

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.

Pediatric Orbital Juvenile Xanthogranuloma: A Case Series and Review of the Literature.

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.

Marginal Full Thickness Blepharotomy for Management of Orbital Compartment Syndrome.

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.

Orbital Giant Cell Reparative Granuloma: A Case Report.

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.

P-ANCA Vasculitis Presenting with Orbital Infiltration.

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.

IgG4-related Orbital Disease Presenting as an Orbital Apex Lesion.

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.

Primary Orbital Respiratory Epithelial Cyst: A Systematic Review and Two Case Reports.

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.

A Case of Lepromatous Leprosy (Lucio's Phenomenon) Presenting as Periorbital Edema.

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.

Secondary Intention Healing After Debridement for Bilateral Periorbital Necrotizing Fasciitis.

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.

Bilateral Superior Ophthalmic Vein Involvement in a Case With Orbital Sarcoidosis.

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.

Erdheim-Chester Disease With Eyelid and Orbital Involvement: A Review of Treatment Modalities at One Institution From 2014 to 2022.

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.

Enlarging Orbital Rim Mass Due to Old Pencil Injury.

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.

An Unusual Case of an Intradiploic Arachnoid Cyst of the Orbit.

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.

Correlation Between Radiologic and Histopathologic Features of Orbital Epidermoid and Dermoid Cysts.

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.

Clinical characteristics and management of orbital apex syndrome: a 10-year multicentre experience.

BACKGROUND: Orbital apex syndrome (OAS) is a condition characterised by lesions within the orbital apex, leading to various ophthalmologic symptoms. This study aimed to analyse the clinical characteristics and treatment strategies of OAS with respect to aetiology. METHODS: This retrospective analysis utilised data from 5 medical institutions between 2013 and 2022. Patients who were diagnosed with OAS were initially enrolled, but patients who failed to follow up at least 1 month were excluded. The prevalence of initial ophthalmologic symptoms and visual improvement after treatment was compared according to aetiology. Factors related to visual improvement were analysed. RESULTS: Among 73 enrolled patients, the leading aetiology was tumours, followed by fungal infections and inflammation. Visual impairment and proptosis were prevalent in tumour-related OAS cases. Inflammation-related OAS exhibited a higher likelihood of painful eye movements and ophthalmoplegia. Ptosis was most frequently observed in fungal infection-related OAS. Notably, fungal infections emerged as the sole significant factor negatively impacting vision progression. In inflammation-related OAS, the time interval between symptom onset and the administration of steroids was longer in patients without visual improvement, even though there was no statistically significant difference. CONCLUSIONS: Tumours were the predominant cause of OAS. Visual impairment was a common manifestation in tumour-related OAS, while fungal infections were strongly associated with a poor visual prognosis. The timely administration of steroids might be helpful for improving vision in patients with inflammation-related OAS. However, further studies are needed to enhance understanding and management of OAS.

[Particulars of sinogenic orbital complications in children]. / Besonderheiten der sinugenen orbitalen Komplikationen bei Kindern.

BACKGROUND: Sinogenic orbital complications in children are relatively rare but critical conditions that require accurate diagnosis and timely appropriate treatment to prevent severe sequelae. OBJECTIVE: This article concentrates on clinical, diagnostic, and therapeutic specifics of sinogenic orbital complications in children. MATERIALS AND METHODS: The work is based on a literature review (PubMed, Google Scholar) and own experience as well as data from the authors' own pediatric population. RESULTS: In children, sinogenic orbital complications are usually secondary due to acute bacterial sinusitis. Correct distinction between pre- and postseptal cellulitis is of utmost importance for correct diagnostic and therapeutic management in order to prevent long-term sequelae. Preseptal cellulitis can be treated conservatively. If there are signs of postseptal involvement, prompt cross sectional imaging will be required. Depending on the severity of the postseptal involvement, surgical treatment may be necessary. CONCLUSION: Recent literature debates three main issues: 1) developing models for different combinations of symptoms that reliably distinguish between pre- and postseptal cellulitis; 2) selection of appropriate cross-sectional imaging (MRI vs. CT); and 3) indications for surgery. All three issues will be discussed in detail in this article.

[Interdisciplinary Management of Orbital Diseases]. / Interdisziplinäre Therapie der Erkrankungen der Orbita.

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.