[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.

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.

Nontraumatic orbital hematoma secondary to labor and childbirth: a case report and review of the literature.

PURPOSE: Non-traumatic orbital hemorrhage without underlying vascular malformations or predisposing conditions is uncommon, and particularly rare in the context of maternal labor. This study combines a novel case report and retrospective review to analyze reported cases and propose insights. METHODS: This study is both a unique case report and literature review examining PubMed publications with articles traced back to original sources through citations for inclusion. Analysis included clinical presentation, visual examination, hematoma characteristics, neuroimaging, management strategies, and outcomes. RESULTS: We present a 37-year-old multigravida woman at 40 weeks gestation who developed acute right-sided proptosis, diplopia, retrobulbar pain, and periorbital edema during the second stage of labor. Computed tomography (CT) revealed a subperiosteal hemorrhage, with subsequent magnetic resonance imaging (MRI) excluding vascular anomalies. Symptoms resolved within two months. Only 14 cases of maternal orbital hematoma associated with labor have been reported. The average age was 28 with 42% (6/14) being primigravid. Including our case, forty percent (6/15) developed symptoms during the second stage of labor, 40% (6/15) immediately postpartum, and 20% (3/15) over 24 hours postpartum. Overall, 33% (5/15) had potentially contributing conditions including coagulopathies, delivery complications, or vascular malformations. Unilateral orbital hemorrhage occurred in 87% (13/15). Surgical intervention was necessary in 13% (2/15). Most (87%, 13/15) underwent observation or medical management with full recovery of symptoms. CONCLUSIONS: Non-traumatic orbital hematomas associated with maternal labor are rare and likely related to increased valsalva during delivery and heightened blood volume in pregnancy. Neuro-imaging and systemic workup are recommended to assess for vascular anomalies or underlying coagulopathies. The overall prognosis is favorable with most having full recovery.

A Patient With Type 1 Diabetes and Acute Rhinosinusitis.

A 41-year-old with type 1 diabetes had generalized weakness, muffled voice, and slurred speech. Neck computed tomography showed soft-tissue gas in the nasopharynx and prevertebral fascia; examination of sinus mucosal samples identified numerous broad, nonseptate right-angled hyphae and fruiting bodies. What is the diagnosis and what would you do next?

Ocular and orbital manifestations in VEXAS syndrome.

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

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.

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.

[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.

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.

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.