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.

[Pediatric orbital Rosai-Dorfman disease: An unusual case]. / Maladie de Rosai-Dorfman à localisation orbito-palpébrale de l'enfant : à propos d'une observation exceptionnelle.

INTRODUCTION: Rosai-Dorfman disease (RDD) is a sinus histiocytosis with massive lymphadenopathy. This rare condition is a benign disease of unknown etiology. Bilateral orbital localization of RDD is rare. OBSERVATION: The authors report the case of a 6-year-old child who presented with bilateral orbital-palpebral masses associated with chronic cervical lymphadenopathy. There were no laboratory signs of inflammation. Serological and tuberculosis screening tests were negative. Histopathological examination of a lymph node biopsy established a diagnosis of Rosai-Dorfman disease. The patient underwent surgical excision of the orbital lesions followed by long-term corticosteroid therapy. A favorable course was observed, with no sign of recurrence after one year of follow-up. CONCLUSION: Rosai-Dorfman disease is very rare in its bilateral orbital presentation. Histopathological diagnosis remains challenging. In Africa, the presence of chronic oculo-palpebral tumor associated with or without cervical lymphadenopathy must raise the suspicion of Rosai-Dorfman disease after ruling out tuberculosis and lymphoma.

Primary Orbital Tuberculosis with Cold Abscess: A Case Report.

Orbital tuberculosis is a rare form of extrapulmonary tuberculosis involving orbital soft tissue, periosteum, bones and lacrimal glands. This is a case report of a 6-year-old male child who presented with swelling of the right upper eyelid. He had normal visual acuity without signs of diplopia or ophthalmoplegia. The tuberculin skin test was reactive and the computed tomography scan showed peripherally enhancing collection with bony erosion and intracranial extension in the extraconal space of the superolateral right orbit. Orbital exploration was done which showed caseous material. The histological examination revealed necrotizing granulomatous tissue. The caseous material on Ziehl Neelsen staining confirmed acid-fast bacilli causing a tubercular abscess. The child is currently on anti-tubercular therapy planned for 12 months. Orbital tuberculosis might or might not be in association with pulmonary tuberculosis and should always be taken into consideration while dealing with chronic inflammatory orbital disease and an orbital mass. Keywords: case reports; orbit; tuberculosis.

[Orbital tumors]. / Tumoren der Orbita.

Orbital tumours include a variety of orbital diseases of different origins. In the case of malignant orbital tumours, early detection is important so that treatment can be initiated promptly. Neuroradiological imaging, in particular magnetic resonance imaging (MRI), plays an important role in the diagnostic of orbital tumours. In adults, lymphoproliferative diseases, inflammations and secondary orbital tumours are most frequently found, whereas in children mostly dermoid cysts, optic gliomas and capillary haemangiomas are found. Optic glioma is a pilocytic astrocytoma and accounts for two thirds of all primary optic tumours. Optic nerve sheath meningiomas mostly affect middle-aged women. In childhood, retinoblastoma is the most common intraocular tumour. This is an aggressive malignant tumour which can occur unilaterally or bilaterally. Based on the imaging findings, differential diagnoses can usually be easily narrowed down using criteria such as age of manifestation, frequency, localisation and imaging characteristics.

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.

Role of Acute Rhinosinusitis in Periorbital Infections in Children.

OBJECTIVE: Periorbital cellulitis in children are commonly caused by acute rhinosinusitis (ARS). This study investigated the association of ARS and the severity of periorbital cellulitis in children. STUDY DESIGN: Retrospective case-control study of children with periorbital cellulitis with ARS versus periorbital cellulitis without ARS. SETTING: Patients were seen at West Virginia University Children's Hospitals between August 2011 to August 2022. METHODS: Patients were divided into cases and controls based on presence or absence of ARS. ARS was defined based on clinical symptoms with objective presence of disease on CT scan. Patients' characteristics, treatment, hospital length of stay, and readmission were collected. RESULTS: The sample consisted of 118 pediatric patients with orbital cellulitis. Patients with ARS were younger than patients without ARS (6.3 vs 8.5, p = 0.025), however there were no sex differences between two groups (p = 0.540). The ARS group had higher incidence of postseptal cellulitis (51.5% vs 9.6%, p < 0.001). As compared to patients without ARS, patients with ARS were more likely to be admitted (p < 0.001), have a longer length of stay (median of 3.5 days vs 0.5 days, p < 0.001), require IV antibiotics (95.3% vs 54.9%, p < 0.001), and require surgical intervention (23.1% vs 5.8%, p < 0.001). Readmission rate was similar between the two groups. CONCLUSION: Children presenting with acute periorbital cellulitis who have ARS tend to have more severe infection requiring higher level of care. ARS should be assessed and incorporated into the plan of care of pediatric patients with periorbital infections.

Survival benefit of exenteration in COVID-19-associated rhino-orbital mucormycosis.

PURPOSE: There has been a sudden increase in the number of rhino-orbital mucormycosis cases, primarily affecting patients recovering from COVID-19 infection. The local health authorities have declared the current situation an epidemic. In this study, we assess the role of exenteration in preventing disease progression and improving survival in patients with rhino-orbital mucormycosis. METHODS: The patients undergoing exenteration were grouped into the exenteration arm and those denying exenteration were grouped into the nonexenteration arm. The patients were followed at 1 month and 3 months. The 6-month survival data were collected telephonically. Continuous data were presented as Mean ± SD/Median (IQR) depending on the normality distribution of data, whereas the frequency with percentages was used to present the categorical variables. Kaplan-Meier survival curves were created to estimate the difference in survival of patients with exenteration in rhino-orbital mucormycosis versus those without exenteration. RESULTS: A total of 14 patients were recruited for our study based on the inclusion and exclusion criteria. All the patients were qualified for exenteration; however, only eight patients underwent exenteration and six patients did not consent to exenteration. At the end of 3 months in the exenteration group, four (50%) patients died. Two patients died within a week of exenteration, whereas two patients died after 2 weeks of exenteration. The deaths in the first week were attributed to septic shock and the deaths happening beyond 2 weeks were attributed to severe meningitis. The Kaplan-Meier survival analysis showed the cumulative probability of being alive at 1 month in the exenteration arm to be 85%, and it decreased to 67% by 53 days and subsequently remained stable until the end of 3 months. CONCLUSION: The Kaplan-Meier survival analysis did not show a survival benefit of exenteration at 3 months and 6 months in COVID-associated rhino-orbital mucormycosis.

Ophthalmology and oculoplastics for the rhinologist.

PURPOSE OF REVIEW: As the management of ophthalmic and sinus disease advances, it is important to stay abreast of all current management options. This involves knowledge of adjacent anatomy and disease processes, and collaboration between multiple specialties. The authors provide a review of relevant ophthalmic and oculoplastic topics for the rhinologist. RECENT FINDINGS: The scope and surgical skills of oculoplastic and rhinologist specialists have grown in conjunction with medical and technological advancements. Familiarity with the presentations of sinus disease in the orbit can decrease the time to disease recognition, and improve morbidity and mortality. Collaborative approaches provide improved access to areas of interest throughout the orbit and sinuses. Multiportal, transorbital, and endoscopic techniques can improve patient morbidity and mortality compared to conventional open approaches. Familiarity with all medical and surgical treatment options can help tailor treatments to patients' needs. SUMMARY: Multidisciplinary approaches to sinus and orbit disease and the incorporation of new surgical technologies can improve patient options and outcomes.

A case report on clinical features, diagnosis, and treatment of rhino-orbito-cerebral mucormycosis.

BACKGROUND: Rhino-orbito-cerebral mucormycosis (ROCM) is an opportunistic pathogenic fungal disease caused by the fungus mucor, and it is a life-threatening fungal infection. The ulceration on the skin of the head and neck, accompanied by rhinitis, headache, orbital inflammation, and eyelid edema, should raise a high suspicion of Mucor infection in diabetic patients with inadequately controlled blood glucose. CASE DESCRIPTION: The clinical data of a patient with ROCM were analyzed retrospectively, and the clinical features were analyzed. The patient was admitted to the hospital with "diabetic hyperosmotic coma" after presenting with fatigue, poor appetite, and disturbances in consciousness as initial symptoms. After improving relevant examinations, controlling underlying diseases, and administering antifungal treatment, the final clinical outcome was death. CONCLUSION: ROCM is more prevalent in patients with uncontrolled diabetes and varied clinical manifestations. The characteristic feature is an eschar-like necrosis of the local skin or mucosa. The gold criteria for diagnosis are pathology and fungal culture; imaging examination does not reveal any specific manifestations. Early diagnosis and effective treatment are the keys.

Study of rhino-orbital-cerebral mucormycosis and its correlates during COVID-19 pandemic in a tertiary eye care institute of central India.

PURPOSE: To evaluate factors associated with the occurrence of ROCM in COVID-19 patients and to compare its related parameters and outcomes between active and recovered COVID-19 groups. METHODS: A total of 35 patients of ROCM associated with COVID-19 (active and post-COVID-19) were included. This is an ambispective study with retrospective medical records review for COVID-19 analysis and prospective assessments of ROCM-associated COVID-19 during the second wave of the COVID-19 pandemic. The demographic data, clinical parameters, and outcome were recorded on MS excel sheet, and various parameters were compared between active and recovered COVID-19 groups. RESULTS: ROCM in recovered COVID-19 group was higher (57.1%) as compared to active COVID-19 (42.9%) (P = 1.00). High occurrence of ROCM was seen in those who had a history of hospitalization due to severity of COVID-19 (n 33, 94.28%), oxygen support (77.14), and received systemic steroids (82.9%). The most common comorbidity was diabetes mellitus (82.9%), and new-onset hyperglycemia was noticed in 17.1% of patients. Exenteration (28.6%) was performed in severe cases who had stage IV ROCM, bilateral, and CNS involvement (RR = 7.2, 95% CI: 2.91 to 18.00). The risk of globe exenteration was 1.35 (0.7-2.29) times higher in recovered COVID-19 group, and mortality was 1.76 (0.72-3.36) times higher in active COVID-19 group. CONCLUSION: Monitored use of systemic steroids and the prompt management of hyperglycemia in COVID-19 patients are important factors for favorable outcomes with reference to globe salvage and life-saving in ROCM associated with COVID-19. Even recovered COVID-19 patients should be observed for persistent hyperglycemia and occurrence of ROCM.

Ocular and orbital manifestations of granulomatosis with polyangiitis: a systematic review of published cases.

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

Bilateral primary orbital xanthogranulomas: A case report and comparison of xanthomatous conditions.

This report describes an unusual and diagnostically challenging case of subcutaneous soft tissue xanthogranulomas of bilateral orbits of a 58-year-old female patient seen in a private oculoplastics practice. Accurate and timely diagnosis is crucial in xanthogranulomatous diseases so that any systemic manifestations can be identified and addressed in a multidisciplinary fashion. Periorbital xanthogranuloma is a frequent early manifestation of adult xanthogranulomatous disease, and its association with life-threatening systemic disease requires accurate diagnosis and prompt work-up. This case describes an otherwise asymptomatic patient who presented with bilateral orbital masses causing visually significant ptosis, initially diagnosed as soft tissue xanthomas, and later identified as xanthogranulomas. It is important for physicians of all fields, from primary care to surgical subspecialty, to be aware that xanthogranulomatous disease may first present as periorbital lesions and/or orbital masses, and that further work-up for vision and life-threatening systemic disease is warranted.

Accuracy of trained technicians in screening orbital and adnexal diseases at rural vision centers in South India.

Purpose: To assess the agreement between the diagnosis made by trained technicians at vision centers (VC) and oculoplasty specialists at the base hospital, in patients referred from VC to the orbit and oculoplasty clinic of a tertiary eye care hospital in south India. Methods: This was a retrospective study that compared the findings of VC technicians and the specialists of the orbit and oculoplasty services in a base hospital. A total of 384 patients referred from 17 VCs between May 2021 and May 2022 were included. The diseases were categorized according to the site of involvement as diseases of the eyelids (43%), diseases of the lacrimal system (37.3%), orbital diseases (15.6), and others (4.1%). The mean age of the patients was 35.9 years and 50.6% were females. Medical records of all referred patients who attended the orbit clinic were analyzed. Results: Of the 384 patients, 378 (98.67%) were confirmed to have o. r: bital and adnexal diseases. There was an overall 80% agreement between the diagnosis made by trained VC technicians and oculoplasty specialists; the kappa coefficient was 0.78 (95% confidence interval [CI]: 0.76 to 0.80), with a P value < 0.001. The agreement was the highest for diseases of the lacrimal system (90.9%, kappa coefficient 0.87), followed by eyelid pathologies (80%, kappa coefficient: 0.77). Of these, 54.8% of patients were managed with surgical procedures. Conclusion: There is good agreement between the findings of VC technicians and oculoplasty specialists. Trained technicians can help in the early detection and referral to higher centers. They also help to make sure adherence to treatment and periodic evaluation, especially in resource-constraint settings.