[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.

Infectious and Inflammatory Processes of the Orbits in Children.

Most primary orbital pathology in children is due to bacterial infection. Radiologists typically encounter these cases to evaluate for clinically suspected postseptal orbital involvement. Contrast-enhanced cross-sectional imaging is important for the detection and early management of orbital infection and associated subperiosteal/orbital abscess, venous thrombosis, and intracranial spread of infection. Benign mass-like inflammatory processes involving the pediatric orbit are rare, have overlapping imaging features, and must be distinguished from orbital malignancies.

Revisiting the orbital complications of acute rhinosinusitis.

OBJECTIVE: The present study revisited three classification systems of orbital complications of acute rhinosinusitis (ARS) (Chandler, Mortimore & Wormald, and Velasco e Cruz & Anselmo-Lima) and observed which of them presented the best clinical applicability. METHODS: Clinical data and CT scan findings of patients with orbital infection were retrospectively collected. To compare the three classification systems, we revised and graded all CT images accordingly, and divided the patients into four groups: Eyelid cellulitis (EC), orbital cellulitis (OC), subperiosteal abscess (SA), and orbital abscess (OA). The groups were compared regarding the presence of sinus opacification, the need for hospitalization and/or surgical treatment, and the presence of further complications/sequelae. RESULTS: 143 patients were included. The median number of sinuses involved in patients in the OC, SA, and OA groups was 2.0. ARS was rarely associated with signs of EC (present in both Chandler's and Mortimore & Wormald's classifications. The hospitalization rate was significantly lower in the EC group compared to the other three groups. Surgery was performed in all cases in the OA group, in 58.1% in the SA group, 19.4% in the OC group, and 12.5% in the EC group (p-value < 0.0001). Complications were present at higher rates in the OA group compared to the other three groups. CONCLUSIONS: ARS was rarely associated with Eyelid Cellulitis. The stratification in the other three groups showed to be clinically relevant. Velasco e Cruz & Anselmo-Lima's classification system proved valid, simple, and effective for categorizing orbital complications of ARS.

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.

Endoscopic-assisted Orbitotomy for Obliteration of Orbital Arteriovenous Fistula Refractory to Endovascular Techniques.

Orbital arteriovenous fistula is a rare acquired disorder. The coincidence of arteriovenous fistula with lymphaticovenous malformation is even rarer. The optimal treatment, thus, is controversial. Surgical approaches vary widely, with associated pros and cons. The purpose of this case report is to describe an orbital arteriovenous fistula in a 25-year-old man with congenital fronto-orbital lymphaticovenous malformation, refractory to endovascular techniques, which was later successfully ablated by a direct endoscopic-assisted orbital approach.

[COVID-19 associated sino-orbital mucormycosis]. / COVID-19-assotsiirovannyi sinoorbital'nyi mukormikoz.

OBJECTIVE: To analyze the dynamics of symptoms in patients with COVID-19 associated sino-orbital mucormycosis. MATERIAL AND METHODS: We describe a series of 13 patients with COVID-19 associated sino-orbital mucormycosis aged 43 to 80 years diagnosed from August to October 2021. All of the patients had a severe disease and required noninvasive ventilation or intubation and administration of dexamethasone. 12 out of 13 patients (92.3%) suffered from diabetes mellitus. Symptoms of fungal infections of the nose and paranasal sinuses appeared in the interval from 7 to 25 days of hospital stay, most often in the second week (from 8 to 12 days). According to clinical and CT features the patients were divided into three groups, combining similar phenotypes of the disease. Group 1 - 1 patient with sinonasal mucormycosis, destruction of the alveolar ridge and the hard palate. Group 2 - 12 patients with sino-orbital mucormycosis. We noted, that in cases of bilateral sinus lesions orbital complications were unilateral in all patients, on the side of more severe lesion. Group 2 was divided into 2 subgroups: subgroup 2a included 2 patients with the superior orbital fissure syndrome: ptosis, proptosis, ophthalmoplegia, periorbital pain, pain or hypoesthesia of half face; subgroup 2b included 10 patients with the orbital apex syndrome, who, in addition to the above symptoms, had loss of vision and conjunctival chemosis. Group 3 - rhino-sino-cerebral mucormycosis. 2 patients from subgroup 2b were transferred to this group due to the intracranial spread of the process and focal neurological symptoms. CONCLUSION: Clinical forms of mucormycosis reflect successive stages of invasive spread of fungi.

Coloboma Accompanying Microphthalmos With Orbital Cyst in a Mother and Child.

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.

The Isabel Differential Diagnosis Generator for Orbital Diagnosis.

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.

Chorio-Retinal Folds Associated With Orbital Cavernous Venous Malformations.

PURPOSE: To determine the incidence of clinically detectable chorio-retinal folds (CRFs) with orbital cavernous venous malformations (OCVMs) and their recovery. METHODS: Retrospective case-note and imaging review, with estimation of odds ratios in relation to the position and volume of the mass. RESULTS: Records for 402 patients were reviewed, 83 (21%) having CRFs. The mean logarithm of minimum angle of resolution acuity was similar with or without CRFs (0.37 and 0.31, respectively; p = 0.46), but induced hyperopia was commoner with CRFs (76% vs. 12%; p < 0.001), exophthalmos greater (4.52 mm vs. 2.97 mm; p < 0.001), eye movement restriction commoner (37% vs. 21%; p = 0.004), and disc swelling more prevalent (42%, vs. 17%; p < 0.001). Orbital cavernous venous malformations with CRFs were almost all intraconal (98%; odds ratio 9.96; p = 0.002), and 93% (77/83) midorbital (odds ratio 6.02; p < 0.001). The median size with CRFs was twice that of those without (3.85 ml vs. 1.92 ml; p < 0.001), and two-thirds OCVMs with folds had volumes >2.5 ml ( p < 0.001). The OCVM was excised in 76 of 83 (92%) of CRF group and 213 of 319 (67%) of those without ( p < 0.001). The postoperative acuity was improved or unchanged in 67 of 76 (88%) eyes with folds, and 184 of 213 (83%) without CRFs ( p = 0.84). The proportion recovering an acuity within 1 Snellen line (or better than) the unaffected side was 80% with CRFs and 77% in their absence ( p = 0.63). Induced hyperopia persisted in 39% of all patients, with the mean being higher with CRFs (2.22D vs. 1.02D; p = 0.017). CONCLUSIONS: CRFs occur in ~25% of mid-intraconal OCVMs. Despite OCVM excision, 39% of operated patients retain significant residual hyperopia (54% if CRFs present before surgery), and 41% of such CRFs remain clinically detectable after surgery (with variable visual impairment). Earlier surgery might, therefore, be advisable in patients with CRFs and/or induced hyperopia.

Giant Lacrimal Gland Cyst in a Pediatric Patient: Case Report and Review of the Literature.

Lacrimal gland cysts are rare clinical entities in the pediatric population. Herein is described a 6-year-old male patient presenting with progressive left upper lid ptosis, found to have a large ipsilateral superotemporal orbital mass. Diagnosis of a giant lacrimal gland cyst was confirmed excisional biopsy. Despite the resolution of mechanical blepharoptosis, the patient maintained visual acuity limitation due to suspected deprivation amblyopia. The pathophysiology and clinical manifestations of lacrimal gland cysts in the pediatric population are reviewed to emphasize the importance of expedited identification and management in this patient cohort.

[Diagnostics and treatment of organic intraorbital foreign bodies]. / Diagnostik und Therapie organischer intraorbitaler Fremdkörper.

BACKGROUND: Orbital injuries with organic foreign bodies are a clinical challenge as they can cause a variety of complications and sometimes lead to irreversible visual impairment. MATERIAL AND METHODS: We report five consecutive cases of patients with organic orbital foreign bodies who presented between 2012 and 2022. Documented parameters include age, gender, time of injury, mechanism of injury, best-corrected visual acuity (BCVA), presence of pain, restriction of motility, performed imaging, location and type of foreign body, treatment and complications. RESULTS: The mean patient age was 61.8 ± 32.3 years and the gender ratio was 4:1 (female:male). In 4 cases the injury had been reported on average 4.6 days (0/14 days min/max) previously. Best corrected visual acuity at first presentation was 0.5 ± 0.2 decimal and after a mean follow-up of 12.7 months (0.5/38 months min/max) at last presentation 0.67 ± 0.3 decimal (p = 0.2). The reason for initial presentation was pain (n = 3) and/or double vision (n = 2). All patients underwent computer tomography (CT). The diagnosis was confirmed in two cases by means of CT. In all five cases the foreign body material could be completely surgically removed, while one patient needed repeated surgery due to retained foreign body material. The organic foreign body material included wood (4) and wax (1). In two cases, a foreign body-induced infection was successfully treated with systemic antibiotics. There were no other complications. CONCLUSION: In orbital trauma a thorough wound exploration must be performed, especially if the mechanism of injury is uncertain and residual foreign bodies or a perforation injury cannot be excluded. The imaging of choice is CT, which may have to be performed again in the postoperative course.

Kimura Disease of the Periorbita and Earlobe in a 15-Year-Old Male: A Case Report and Review of the Literature.

PURPOSE: The authors describe a case of Kimura disease (KD) affecting the lacrimal gland, the ipsilateral periorbita, and the contralateral earlobe. The authors also review the literature for characteristics and outcomes of KD affecting the periorbita or earlobe. METHODS: The authors review retrospectively a single chart and abstract data from the English language literature. RESULTS: A 15-year-old Samoan male presented with a left upper eyelid mass and later the contralateral earlobe and ipsilateral periorbita. Surgical resection was partially effective and normal vision and eye motility were maintained. Histopathology was consistent with KD. Thirty-seven cases of periorbital KD were identified in the literature. The average age of patients with periorbital KD was 31.9 years (standard deviation: 17.8 years), with 81.1% (30/37) of patients being male and 69.4% (25/36) Asian. Over half of periorbital KD patients had lacrimal gland involvement (51.3%; 19/37). Fifteen patients were initially treated with surgery; 3 (20%) had recurrence of the disease. Another 15 patients were initially treated with corticosteroids; 12 (80%) had recurrence of the disease. Ophthalmologic data, when reported, demonstrated that, most patients had no visual (77.8%; 14/18 patients) or motility disturbances (82.4%; 14/17 patients) but most had proptosis 71.4% (10/14). Only 4 cases of earlobe involvement in KD were identified, all in women. CONCLUSIONS: In the literature, orbital KD was rare but often affected the lacrimal gland and caused proptosis, commonly in young adult Asian males. Vision and extraocular motility were usually normal. Earlobe involvement was very rare. This unique case of KD that affected the orbit and contralateral earlobe corroborates the prior literature that KD is prone to recurrence but may not adversely affect vision or extraocular motility.

Erdheim-Chester disease and vemurafenib: a review of ophthalmic presentations and clinical outcomes.

PURPOSE: To provide a comprehensive review of ocular and orbital manifestations of Erdheim-Chester Disease (ECD) and compare clinical outcomes with vemurafenib (INN) to historical treatments (HT). Primary outcomes are ophthalmic findings on presentation, changes in visual acuity, and mortality rate. Secondary outcomes include the progression of ocular findings, systemic involvements, and treatment modalities. METHODS: All published literature from January 1983 to March 2021 was searched for ophthalmic manifestations of ECD. Clinical outcomes following HT were collected and compared with INN. RESULTS: Forty-seven patients with ECD and ophthalmic presentations were identified. The mean age was 49.6 years (SD = 15.0). Proptosis (65.6%) and extraocular muscle restrictions (42.5%) were the most common presenting signs. Of 41 (87.2%) patients with orbital masses on radiologic examination, 90.2% were bilateral, and 53.7% were located in the intraconal space. Ophthalmic examination was significant for xanthelasma (27.2%), optic disc edema (34.0%), and subretinal changes (21.3%). Common treatments were systemic steroids (76.6%), interferon-α (17.0%), and cyclophosphamide (14.9%). INN was less commonly used (12.8%). The mean change in logMAR visual acuity declined with HT (29.9%) but improved with INN (79.1%) (p > 0.05). The proportion of eyes with complete vision loss increased after HT (p < 0.05). The overall mortality rate was 27.7% and notably higher in the HT group (29.3%) when compared to the INN group (16.7%) (p > 0.05). CONCLUSION: ECD presents with many ophthalmic manifestations. Although the intraocular treatments remain controversial, INN should be highly considered in treating orbital ECD patients with BRAF-V600E mutations to prevent and reverse vision loss.

A study of rhino-orbito-cerebral mucormycosis with COVID-19: A new challenge in North West of Rajasthan.

Background: Mucormycosis is a life-threatening fungal disease in immunocompromised patients. There has been increase in the number of mucormycosis associated with COVID-19 patients in second wave. Now country battle with both COVID-19 and mucormycosis. An invasive mucormycosis infection has been a significant burden in India after COVID-19. It has been recently emerged a notifiable disease by the Rajasthan government. Our aim is to develop awareness regarding the importance of early detection and treatment of mucormycosis with COVID-19 and reduce the morbidity and mortality. Materials and Methods: This is a Prospective longitudinal study including 34 patients diagnosed with acute invasive fungal infection by contrast enhancement magnetic resonance imaging studies of paranasal, orbit and brain or nasal biopsy for KOH/culture. Diagnosis is made through routine blood tests, biopsy, and radiological imaging. The patients taken for the study were COVID-19 reverse transcription-polymerase chain reaction positive or recent post COVID-19 (within 15 days) or symptoms of COVID-19 with bilateral pneumonitis. The study was conducted with 34 patients admitted to the department of medicine with mucormycosis within a month may 2021. Results: A total of 34 patients with a mean age of 50.92 years old and male female ratio 24/10 (70.5/29.41) were included in this study. The most common comorbidity was diabetes mellitus (23 patients 67.64%). Nine patients were newly diagnosed or recent onset of diabetes with or after COVID-19 infection. Twenty-four (70.58%) patients were COVID-19 positive or recent (within 15 days) history of COVID-19 positive. Seven (20.58) patients had the history of steroid as a treatment during COVID-19 and 5 (14.70) patients was on oxygen inhalation. One (2.94%) patient was fully vaccinated, and 5 (14.70) patients had the history of steam inhalation. The most common involvement was naso-orbital mucormycosis found in 28 patients (82.35%) followed by nasal-and orbital 26 (76.47) and 18 (52.94), respectively. Naso-Orbito-Cerebral was seen in 16 (47.05) patients. The more common reported symptoms and signs were headache (76.47), facial numbness (64.70), Nasal discharge (52.94), and ophthalmoplegia (52.94). Cranial nerve involvement was seen in 10 patients (facial palsy in 8 patients and bulbar palsy in 2 patients). Total mortality was 7 (7/34 20.58%). Conclusion: COVID-19 infection associated with the wide range of invasive mucormycosis. Early diagnosis and Clinical suspicion of acute invasive fungal sinusitis among COVID-19 patients is essential for better outcomes and higher survival.

Résumé Contexte: La mucormycose est une maladie fongique mortelle chez les patients immunodéprimés. Il y a eu une augmentation du nombre de mucormycose associée aux patients Covid - 19 en deuxième vague. Maintenant, le pays se bat contre le Covid-19 et la mucormycose. Une infection invasive en mucormycose a été une charge significative en Inde après Covid - 19. Il a récemment émergé une maladie notifiable du gouvernement du Rajasthan. Notre objectif est de sensibiliser à l'importance de la détection et du traitement précoce de la mucormycose avec Covid-19 et de réduire la morbidité et la mortalité. Matériaux et méthodes: Il s'agit d'une étude longitudinale prospective comprenant 34 patients diagnostiqués avec une infection fongique invasive aiguë par un contraste d'imagerie magnétique des études d'imagerie par résonance magnétique de biopsie paranasale, en orbite et au cerveau ou nasale pour le KOH / la culture. Le diagnostic est posé par des tests sanguins de routine, une biopsie et une imagerie radiologique. Les patients pris pour l'étude ont été la réaction en chaîne de la transcription inverse de Covid-19, la réaction en chaîne de polymérase positive ou le post-COVID-19 récent (dans les 15 jours) ou les symptômes de Covid-19 avec une pneumonite bilatérale. L'étude a été menée avec 34 patients admis au Département de médecine avec mucormycose dans un mois en mai 2021. Résultats: Un total de 34 patients avec un âge moyen de 50,92 ans et un rapport féminine masculin 24/10 (70,5 / 29,41) ont été inclus dans cette étude. La comorbidité la plus courante était le diabète sucré (23 patients 67,64%). Neuf patients ont été récemment diagnostiqués ou un début récent du diabète avec ou après l'infection à Covid - 19. Vingt-quatre (70,58%) patients étaient des antécédents de Covid - 19 positifs ou récents (dans les 15 jours) de Covid - 19 positifs. Sept (20,58) patients avaient des antécédents de stéroïde comme traitement pendant les patients COVID-19 et 5 (14,70) étaient sous inhalation d'oxygène. Un patient (2,94%) a été entièrement vacciné et 5 (14,70) patients avaient des antécédents d'inhalation de vapeur. L'atteinte la plus courante était la mucormycose naso-orbitale trouvée chez 28 patients (82,35%), suivie respectivement par l'orbital nasal et orbital 26 (76,47) et 18 (52,94). Le naso - orbito-cervebral a été observé chez 16 (47,05) patients. Les symptômes et les signes rapportés les plus courants étaient des maux de tête (76,47), un engourdissement facial (64,70), une décharge nasale (52,94) et une ophtalmoplégie (52,94). Une atteinte du nerf crânien a été observée chez 10 patients (paralysie faciale chez 8 patients et paralysie bulbaire chez 2 patients). La mortalité totale était de 7 (7/34 20,58%). Conclusion: Infection Covid - 19 associée à la large gamme de mucormycose invasive. Le diagnostic précoce et la suspicion clinique de sinusite fongique invasive aiguë chez les patients COVID-19 sont essentiels pour de meilleurs résultats et une survie plus élevée. Mots-clés: Covid - 19, fongique, invasive, nasal, rhinocéros orbital cerebral.

Rhino-orbital mucormycosis in a patient with no susceptibility following P.vivax malaria infection-a case report.

BACKGROUND: Mucormycosis is a potentially lethal, angioinvasive fungal infection caused by the Mucoracea family comprising Mucor, Rhizopus, and Absidia species. It is commonly associated with uncontrolled diabetes mellitus, the use of corticosteroids, immunosuppressive drugs, and Covid-19 infection. The occurrence of mucormycosis in an immunocompetent patient is rare. Also, only a few case reports have been published where patients developed mucormycosis with associated malarial infection. CASE PRESENTATION: A young female presented with a 3-weeks history of painful swelling and outward protrusion of the right eye with complete loss of vision. She had a history of P.vivax malaria two weeks before her ocular symptoms. On ocular examination, there was proptosis and total ophthalmoplegia with loss of corneal sensations in the right eye. Hematological examination revealed normocytic normochromic anemia and thrombocytopenia. MRI was suggestive of right-sided pansinusitis and orbital cellulitis with right superior ophthalmic vein thrombosis and bulky cavernous sinus. Nasal biopsy was negative for fungal culture. An emergency surgical debridement of all the sinuses was done with right orbital exenteration. Histopathology confirmed the diagnosis of mucormycosis and the patient improved post-operatively on systemic antifungals. CONCLUSION: Such an association of mucormycosis with malaria infection is rarely reported in the literature and is hypothesized to be a result of immunosuppression caused by malaria. Also, emphasis is laid upon having a high index of suspicion for fungal infection in the setting of pansinusitis even if the risk factors are absent. We hereby report a case of rhino-orbital mucormycosis following P.vivax malaria in a 20-year-old female with anemia and thrombocytopenia.

Unusual association of orbital tumour and rhino-orbital-cerebral mucormycosis.

In developing tropical countries, rhino-orbital-cerebral mucormycosis has been a cause of severe morbidity and mortality during the COVID-19 pandemic. Classically, it develops as an aggressive angioinvasive destruction of nasal, orbital and cerebral involvement. Blindness is a major disabling complication. The association of mucor in cancer is linked with immunosuppression caused by radiation and/or chemotherapy. In this case report, we tried to explore the diverse possibilities of neck swelling, nasal discharge, ocular swelling and dimness of vision in a teenage boy. Rhabdomyosarcoma is a rare tumour of the soft tissue, connective tissue or bone. This type of unusual association or coexistence of rhabdomyosarcoma with mucormycetes is rarely seen in literature.

[Rare association of subperiosteal hematoma of the orbit with frontal extradural hematoma: a case report]. / Association rare d'un hématome sous-périosté de l'orbite et d'un hématome extradural frontal: à propos d'un cas.

Subperiosteal haematoma of the orbit associated with frontal extradural hematoma is very rare. We here report a case treated in our Department with a literature review. The study involved a 15-year-old boy, victim of head injury from white weapon, a week before his admission to the emergency room. He had inflammatory and painful exophthalmos on the left side, associated with left ophthalmoplegia and blindness. Brain scanner showed left frontal extradural hematoma associated with subperiosteal hematoma of the ipsilateral orbit. Frontal craniotomy associated with fracture orbitotomy allowed evacuation of the extradural hematoma, and then, of the subperiosteal hematoma. Patient´s outcome was favorable. Simultaneous occurrence of frontal extradural hematoma and subperiosteal hematoma of the orbit is extremely rare. Generally, attention is drawn by exophthalmos and visual disturbances. Emergency brain scan without contrast agent injection can be used to make a diagnosis. Prognosis depends on visual function, then adequate management helps to safeguard the eye and vision.