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Ophthalmoplegia, proptosis, and orbital congestion are the most frequent findings. It is caused by lesions of the cavernous sinus. Parasellar lesions including tumors, carotid artery aneurysms, and carotid-cavernous fistulas have been described.

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Foix Syndrome; Jefferson Syndrome; Foix-Jefferson Syndrome.

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Approximately 5% of all ophthalmoplegias are the result of cavernous sinus syndrome.

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No genetic background.

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The cavernous sinuses are paired. They contain the oculomotor nerves, carotid arteries, and their sympathetic plexus and are traversed by the ophthalmic branch of the trigeminal nerve. Tumors (primary or secondary), carotid artery aneurysms, carotid-cavernous fistulas, and inflammatory processes are the main causes of this disorder.

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The syndrome can be diagnosed clinically. Funduscopy shows optic disc edema and retinal hemorrhages, however, diagnostic imaging and laboratory studies are required to establish the nature of the lesion.

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On examination, patients have ophthalmoplegia, orbital congestion, and proptosis. Cranial nerve palsies may be isolated or comprise a combination of third, fourth, and sixth cranial nerve palsies. Ophthalmoplegia may be painful. Ocular pulsation suggests a vascular fistula. There may be conjunctival congestion and arterialization of the conjunctival veins, diminished sensation in the ophthalmic branch of the trigeminal nerve, and decreased or absent corneal reflex. The pupil on the affected side is typically in mid-position and nonreactive to light.

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Patients should be assessed for raised intracranial pressure and potential endocrinopathy (both, hormonal overand underproduction) associated with tumors in or around the sella turcica. Serum electrolyte and glucose levels should be measured. Where applicable, treatment of the endocrinopathy should be initiated prior to surgery.

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Main anesthesia concerns are raised intracranial pressure (occasionally) and endocrine effects with associated organ dysfunction. Maintain a cerebral perfusion pressure of at least 60 mmHg, prevent seizures and fever, and adjust ventilation to aim for low normocapnia to mild hypocapnia. However, in the presence of vascular lesions, rapid increases in blood pressure must also be avoided.

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In the presence of increased intracranial pressure, drugs that increase cerebral blood flow should be avoided or used only at low concentrations/doses.

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Tolosa-Hunt Syndrome: Refers to a cavernous-sinus syndrome caused by an idiopathic inflammatory lesion, an abnormal autoimmune response, or tumors/metastases in the area of the cavernous sinus. Symptoms most commonly occur at the beginning of the fifth decade of life and include chronic and severe (often unilateral) headache often preceding ophthalmoplegia. Mild fever, double vision, exophthalmos, ptosis, vertigo, chronic fatigue, and arthralgia may occur. Steroids have been used successfully; however, spontaneous remission is common (except when caused by tumors/ metastases), as is recurrence. No sexual predilection has been reported.

Biousse V, Mendicino ME, Simon DJ, et al: The ophthalmology of intracranial vascular abnormalities. Am J Ophthalmol 125:527, 1998.  [PubMed: 9559739]
Keane JR: Cavernous sinus syndrome: Analysis of 151 cases. Arch Neurol 53:967, 1996.  [PubMed: 8859057]
Van Overbeeke JJ, Jansen ...

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