Cases reported "Exophthalmos"

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1/13. 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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2/13. Angiomatoid melanoma: a novel pattern of differentiation in invasive periocular desmoplastic malignant melanoma.

    A case of locally invasive, long-standing desmoplastic and amelanotic malignant melanoma is described in an 84-year-old man. Histologic examination of the involved periorbital tissue showed neoplastic foci exhibiting a novel pattern reminiscent of microvascular proliferation. These regions were characterized by malignant, S-100-positive tumor cells lining vessel-like spaces in transverse sections and forming tubuler-like structures in longitudinal sections. Recent data indicate that melanoma cells may express genes and patterns of differentiation in vitro akin to endothelial cells. Because angiosarcoma often involves facial and scalp skin of elderly individuals, awareness of angiomatoid differentiation in melanoma has important diagnostic implications. HUM PATHOL 31:1520-1522.
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3/13. Posttraumatic carotid-cavernous sinus fistula.

    BACKGROUND AND OBJECTIVES: Posttraumatic carotid-cavernous sinus fistula is a rare complication of maxillofacial trauma and is seldom discussed in the literature. Motor vehicle accidents, falls, and other crush injuries contribute to the incidence of basilar skull fractures and the formation of fistulae. When injuries occur in the vessel wall, the carotid artery has the potential to fill the low-pressure cavernous sinus. The symptoms include chemosis, proptosis, pulsating exophthalmos, diplopia, ophthalmoplegia, orbital pain, audible bruits, and blindness. methods AND MATERIALS: The conventional treatments include carotid ligation and embolization. These techniques have often proved to be ineffective. A new method--the occlusive balloon technique--has been developed and is described in this article. A clinical case is used to illustrate the procedure. RESULTS AND/OR CONCLUSIONS: Utilization of balloon catheters provides a minimally invasive technique to treat patients, without significant morbidity or mortality. The procedure is found to be successful and predictable.
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4/13. Combined central retinal artery and vein occlusion from orbital inflammatory pseudotumour.

    Progressive, painful blurred vision and proptosis developed in the left eye of a 32-year-old man over an 8-day period. On initial neuro-ophthalmic evaluation the visual acuity in the left eye was no light perception. erythema of the periorbital skin, 5 mm of proptosis, and ophthalmoplegia were noted on the left side. Funduscopy revealed occlusion of the central retinal artery and central retinal vein. Computed tomography and magnetic resonance imaging revealed marked distension of the left optic nerve and enhancement of the contents within the left orbit, with clear paranasal sinuses. The diagnosis of orbital inflammatory pseudotumour was made and the orbital signs improved rapidly with 80 mg of prednisone per day. Combined occlusion of the central retinal artery and vein is a rare complication of orbital inflammatory pseudotumour. It is postulated that marked distension of the optic nerve caused mechanical compression of the central retinal vessels leading to the vascular occlusions.
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5/13. Isolated neurofibroma of the orbit with extensive myxoid changes: a clinicopathologic study including MRI and electron microscopic findings.

    Isolated orbital neurofibroma (INO) without systemic neurofibromatosis is rare. Previous reports including MRI with elaboration of the ultrastructural features of the cellular and extracellular components in INO do not exist. A 44-year-old male presented with painless, slowly progressive proptosis (6 mm) and downward displacement (3 mm) of the left eye of two years duration. MRI revealed a suprabulbar mass hypointense to fat, isointense to brain on T1-weighted images with small hyperintense areas following contrast enhancement. T2-weighted images showed a predominantly hyperintense mass with a few hypointense islands. The lesion was excised using an anterior superior transseptal orbitotomy. Histopathologically, large areas of a paucicellular myxoid stroma containing mucopolysaccharides made up approximately three-quarters of the mass. Focal hypercellular areas showed marked collagen deposition. blood vessels were distributed at the periphery of the lesion and in association with nodules of nerve sheath cells. Ultrastructurally, perineural and fibroblast-like cells were loosely arranged in a myxoid stroma. schwann cells were embedded in a dense collagenous matrix. Variable differentiation of schwann cells, perineural cells and fibroblast-like cells associated with a more solid (collagenous) or loose (myxoid) extracellular matrix may determine the characteristic, though not specific, MRI features in isolated neurofibromas of the orbit.
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6/13. Mechanisms of visual loss in severe proptosis.

    Vision loss in orbital hypertension secondary to sudden space-occupying lesions is usually attributed to one of three causes: central retinal artery occlusion, direct compressive optic neuropathy, or compression of optic nerve vasculature. Accepted modes of decompressive therapy include lateral canthotomy and cantholysis; drainage of localized orbital air, hematoma, or abscess; and bony wall decompression. Five cases are presented in which orbital hypertension caused severe proptosis with traction on the optic nerve and tenting of the posterior globe. Another mechanism contributing to visual loss is proposed in these cases: ischemic optic neuropathy due to stretching of nutrient vessels. In these cases, rapid posterior decompression should theoretically be favored to reduce orbital pressure and relieve traction on the optic nerve vasculature.
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7/13. orbital pseudotumor, differential diagnosis.

    A 66-year-old white male presented with complaints of redness of his left eye, blurred vision, lid edema, and an intermittent throbbing pain above his left eye for 4 days. Significant clinical findings included mild hyperemia and edema of the left upper lid, marked engorgement and tortuosity of the conjunctival and episcleral vessels, mild chemosis, and extreme limitation of the extraocular movements of the left eye in all directions of gaze, especially adduction. This eye also showed a measurable proptosis with positive retropulsion. Pupillary responses and color vision were normal in both eyes. A CT scan revealed an enlarged medial rectus muscle, including its tendinous insertion with a normal cavernous sinus. On the basis of the clinical findings and the CT scan, a diagnosis of orbital pseudotumor was made. The patient was treated with high dose systemic prednisone, which resulted in a dramatic improvement of symptoms and signs.
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8/13. Ultrasonic examination of orbital vasculature.

    patients with orbital varices form a diagnostically important group of those referred for ultrasonic study of orbital proptosis. Two cases are presented in which diagnosis of dilated orbital blood vessels was established by B scan ultrasonography. The echographic appearance of normal and dilated orbital vessels is discussed.
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9/13. Arteriovenous shunts between dural branches of the carotid artery and the cavernous sinus.

    Three cases with dural arteriovenous shunts of the cavernous sinus are reported. The clinical signs usually were mild and included frontal headache, dilated conjunctival veins, exophthalmos, bruit, and oculomotor nerve palsy. Arterial contributions to the fistulae arose from meningeal branches of the internal and external carotid arteries. The precise diagnosis was made by selective angiography supplemented by subtraction and magnification techniques. The distal occlusion of the feeding arteries close to the shunt offers advantages when compared with conventional vessel ligation because of a more effective exclusion of vascular abnormalities from the circulation.
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10/13. Direct carotid-cavernous sinus fistulas.

    1. Direct carotid-cavernous sinus fistulas usually result from multi-trauma or head injuries. These patients are more likely to be seen at trauma based hospitals. 2. Ocular manifestations of direct carotid-cavernous sinus fistulas include exophthalmos, swollen eyelids, ocular pulsation, pulsating exophthalmos, conjunctival chemosis, arterialization of conjunctival vessels, exposure keratopathy, a bruit over the affected eye (heard by the patient as a swishing sound), diplopia, visual loss, and ophthalmoscopic abnormalities that include moderate dilation of retinal veins, optic disc swelling, and intraretinal hemorrhage. 3. The ultimate diagnostic test for direct carotid-cavernous sinus fistulas is cerebral arteriography of both the internal and external carotid arteries. 4. The optimal treatment of a direct carotid-cavernous sinus fistula is closure of the abnormal arteriovenous communication with preservation of internal carotid artery patency. Detachable, flow-guided balloons are presently used to close most direct carotid-cavernous sinus fistulas.
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