Cases reported "Orbital Diseases"

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1/18. Clinical findings in a patient with spontaneous arteriovenous fistulas of the orbit.

    PURPOSE: To report clinical and radiologic findings of a patient with spontaneous arteriovenous fistulas of the orbit. METHOD: Case report. RESULTS: A 73-year-old woman was initially examined with a 1-year history of mild proptosis of the right eye. She had no history of trauma. Neuro-ophthalmologic examination disclosed dilatation of conjunctival vessels, increased intraocular pressure, mild proptosis and bruit in the right eye, and ocular signs suggestive of carotid-cavernous sinus fistulas or orbital arteriovenous malformations. The patient exhibited dilation of the superior ophthalmic vein in enhanced computed tomography of the orbit. Selective cerebral angiography disclosed communications between branches of both ophthalmic and facial arteries and the superior ophthalmic vein in the orbit. CONCLUSION: Arteriovenous fistulas of the orbit must be considered in the differential diagnosis of carotid-cavernous sinus fistulas and arteriovenous malformations, although they are quite rare.
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2/18. Sickle cell disease with orbital infarction and epidural hematoma.

    Although bone infarction is a common feature in sickle cell disease, the involvement of the orbit is an unusual complication. Intracranial bleeding is another uncommon and serious complication. Few cases of orbital infarction alone have been reported. We report imaging findings (CT, bone scan, MRI) in a 16-year-old boy with sickle cell disease with orbital infarction and epidural hematoma. The precise cause of epidural hematoma is not well known, but it is probably related to vaso-occlusive episodes and the tearing of small vessels.
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3/18. Cadaveric study of blood supply to the lower intraorbital fat: etiologic relevance to the complication of anaerobic cellulitis in orbital floor fracture.

    BACKGROUND AND PURPOSE: Although orbital fractures are common, orbital cellulitis rarely develops following orbital fracture. We hypothesized that compromise of the blood supply to the intraorbital fat during orbital floor fracture is responsible for this condition. The purpose of this study was to determine whether or not the lower intraorbital fat is supplied by a branch of the infraorbital artery along the orbital groove or canal on the orbital floor. MATERIALS AND methods: We dissected 14 orbits from seven fixed human cadavers and 12 orbits from six fresh cadaver heads following dye injection into the maxillary artery. The sites of dye-filled vessels branching from the infraorbital artery supplying the lower intraorbital fat were measured and plotted on a two-dimensional orbital floor graph. RESULTS: A main branch of the infraorbital artery rose through the medial orbital floor to supply the lower intraorbital fat in all of the cadaver orbits. The sites of the branching point of the vessel ranged from 0 to 5 mm (mean, 2.2 mm; n = 14) medial to the line connecting the infraorbital foramen and the infraorbital groove. The shortest distance measured from the branching point to the orbital rim ranged from 3 to 20 mm (mean, 14.1 mm; n = 14). This suggests that if orbital fracture were to occur around the infraorbital groove or canal, this vascular pedicle would be in danger of being incarcerated by bone fragments. CONCLUSION: Our cadaveric investigation revealed that the lower intraorbital fat is supplied by a branch of the infraorbital artery along the infraorbital groove or canal on the orbital floor. This finding suggests that compromised blood supply to the intraorbital fat may cause anaerobic cellulitis or enophthalmos.
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4/18. Case report. Mycotic arteritis due to aspergillus fumigatus in a diabetic with retrobulbar aspergillosis and mycotic meningitis.

    A 74-year-old man with diabetes mellitus type II, retinopathy and polyneuropathy suffered from exophthalmus, ptosis and diplopia. magnetic resonance imaging and computer tomography showed a space-occupying process in the right orbital apex. An extranasal ethmoidectomy accompanied by an orbitotomia revealed the presence of septated hyphae. aspergillus fumigatus was grown from the tissue. After surgical removal of the fungal masses, therapy with amphotericin b (1 mg kg(-1) body weight) plus itraconazole (Sempera, 200 mg per day) over 6 weeks was initiated. Five months later the patient's condition deteriorated again, with vomiting, nausea and pain behind the right eye plus increasing exophthalmus. Antifungal therapy was started again with amphotericin b and 5-fluorocytosine. neutropenia did not occur. The patient became somnolent and deteriorated, a meningitis was suggested. Aspergillus antigen (titre 1:2, Pastorex) was detected in liquor. Anti-Aspergillus antibodies were not detectable. Both the right eye and retrobulbar fungal masses were eradicated by means of an exenteratio bulbi et orbitae. However, renal insufficiency and an apallic syndrome developed and the patient died. At autopsy, a mycotic aneurysm of the arteria carotis interna dextra was detected. The mycotic vasculitis of this aneurysm had caused a rupture of the blood vessel followed by a massive subarachnoidal haemorrhage. In addition, severe mycotic sphenoidal sinusitis and aspergillosis of the right orbit were seen, which had led to a bifrontal meningitis.
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5/18. A red eye and then a really red eye.

    A 90-year-old woman presented with a 4-week history of a presumed infectious conjunctivitis resistant to topical antibiotic medications. Examination revealed tortuous, dilated conjunctival vessels in the right eye, retinal hemorrhages, and an orbital bruit suggestive of a carotid-cavernous sinus fistula (CCF). While awaiting a magnetic resonance imaging study, she returned to the clinic the next day with a painful, swollen right eye and an intraocular pressure of 69 mm Hg. A cerebral arteriogram confirmed a direct CCF. Because of the tortuosity of the systemic vascular anatomy, a right carotid artery cut-down with balloon occlusion was performed with successful closure of the fistula and prompt resolution of the orbital congestion. This case illustrates the spectrum of subtle to conspicuous ocular manifestations that can be seen in patients with CCF and its potential to present as an emergency. CCF should be included in the differential diagnosis of an "atypical" red eye. Recognition of arteriolized conjunctival vessels and auscultation of an orbital bruit raises the possibility of a CCF, requiring prompt diagnostic studies.
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6/18. Refractory Wegener's granulomatosis responds to tumor necrosis factor blockade.

    Wegener's granulomatosis is a systemic necrotising vasculitis of small vessels that leads to severe impairment of affected organ systems. Conventional treatment is based on immunosuppression with a combination of steroids, cyclophosphamide, azathioprine or methotrexate over a prolonged time course. Early recurrence or disease refractory to therapy often results in a fatal outcome. As in other inflammatory disorders, tumor necrosis factor (TNF) plays an early and crucial role in progression of disease activity. We report on a patient with severe orbital Wegener's granulomatosis who developed acute renal failure despite intense conventional immunosuppression with cyclophosphamide and steroids. To stop vasculitic activity, by disrupting the autoimmune inflammatory cascade, a TNF-blocking antibody (Infliximab) was administered six times in a six-month period at 3 mg/kg body weight. Conventional immunosuppressive therapy with steroids and cyclophosphamide was continued, the latter being changed to azathioprine after three months. The first infusion of TNF antibody induced improvement of renal function, which continued throughout the course of therapy. The modification of diet in renal disease-glomerular filtration rate (MDRD-GFR) increased from 15.3 ml/min/1.73 m2 before the start of TNF-blockade to 55.5 ml/min/1.73 m2 after six months of therapy. serum creatinine levels, proteinuria and cANCA titer decreased concomitantly. Clinical remission of Wegener's granulomatosis was induced without any major adverse events. A slight flare of orbital inflammation was successfully treated with an increased dose of azathioprine. Thus, in this case of refractory Wegener's granulomatosis TNF-blockade by monoclonal chimeric TNF-antibody (Infliximab) served as an effective tool to rescue kidney function and induce clinical remission.
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7/18. Rhinoorbital mucormycosis secondary to rhizopus oryzae: a case report and literature review.

    mucormycosis is a form of fulminant invasive fungal infection of the sinonasal tract that often extends to the orbit, brain, palate, and skin. It is caused by members of the order mucorales, and it is considered to be the most fatal fungal infection known to man because it is rapidly disseminated by the blood vessels. It is most commonly associated with diabetic ketoacidosis, hematologic malignancies, acquired immunodeficiency syndrome, and immunosuppressive therapy. This rare opportunistic infection exists in many forms, the most common of which is rhinocerebral mucormycosis. Treatment includes aggressive surgical debridement of the necrotic tissue combined with systemic antifungal therapy. In this case report, we describe the successful management of rhinoorbital mucormycosis, a subtype of the rhinocerebral variety, secondary to rhizopus oryzae that developed in a patient with lymphoma. We review the diagnostic work-up and discuss the literature with respect to the presentation, pathophysiology, management, and outcome of the disease.
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8/18. Orbitoethmoid aneurysmal bone cyst. Case report and literature review.

    Aneurysmal bone cyst is a benign, vascular, variable growing and expansive lesion that can occur in any part of the skeletal system, but mainly in long bones and vertebrae. We present a case of orbitoethmoid aneurysmal bone cyst in a 62-year-old female presenting epiphora. Nasal endoscopy was normal. Sinus CTscan revealed an expansive mass in the right ethmoid sinus extending and destroying the right lamina papiracea. The tumor was completely resected through paralateral rhinotomy. Histological analysis showed fibrous septa containing multinucleated giant cells and bone tissue surrounding blood vessel lumens, bordered by endothelial cells. These findings are characteristic of aneurysmal bone cysts. After 30 months of postoperative follow-up the patient remains disease-free and asymptomatic.
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9/18. Orbital eosinophilic angiocentric fibrosis case report and review of the literature.

    OBJECTIVES: To report a patient with a rare case of orbital eosinophilic angiocentric fibrosis (EAF) and to review the literature. DESIGN: Interventional case report. methods: A 61-year-old man presented with a 6-week history of right periorbital edema and painless proptosis. Examination revealed a nonaxial proptosis, lateral globe displacement, and mild limitation in right eye adduction. MAIN OUTCOME MEASURES: Clinical course and radiological and histological findings. RESULTS: Orbital imaging revealed a right medial orbital mass with involvement of middle ethmoidal air cells. An orbital biopsy of the mass demonstrated an inflammatory infiltrate with a marked eosinophilic component, onion skinning of vessels, and surrounding fibrosis. The diagnosis of orbital EAF was made. There was no response to a 3-month treatment course with systemic steroids, but the patient did not want any further surgical interventions. CONCLUSION: Although orbital EAF is rare, ophthalmologists need to be aware of this entity, as it may invade the orbit from the sinonasal tract or present as a localized orbital mass. The presence of even minimal sinus involvement and the characteristic histopathology are useful in establishing the correct diagnosis.
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10/18. Recurrent bilateral dacryocystoceles in Wegener's granulomatosis: a rhinologic perspective.

    Wegener's granulomatosis (WG) is a rare, idiopathic, systemic vasculitis of small vessels that manifests in multiple organ systems. Otorhinolaryngic manifestations of this disease include recurrent sinusitis and relapsing polychondritis. Periocular involvement is also a well-documented location of Wegener's disease. We present the case of a 13-year-old girl with severe WG who developed multiple recurrent orbital infections. She underwent multiple incision and drainage surgeries of each orbit and multiple courses of intravenous antibiotics. The patient persistently reaccumulated purulence in her nasolacrimal duct system and was referred to an oculoplastic surgeon for evaluation of these recurrent infections. The diagnosis of dacryocystitis as a complication of WG was made. This unique case represents a patient with severe WG developing bilateral dacryocystitis requiring bilateral dacryocystorhinostomies.
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