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At a glance

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

Synonyms

Foix Syndrome; Jefferson Syndrome; Foix-Jefferson Syndrome.

History

One of the first who associated intracavernous aneurysms with Cavernous Sinus Syndrome (CSS) was the American physician Roberts Bartholow (1831-1904). In 1921, the French neurologist Charles Foix (182-1927) was one of the first to recognize that unilateral ophthalmoplegia can be caused by CSS. Extensive work published in 1938 and later in 1953 by the English neurologist and neurosurgeon Geoffrey Jefferson (1886-1961) further elucidated the etiology of CSS, particularly the association with internal carotid artery aneurysms.

Incidence

Approximately 5% of all ophthalmoplegias are the result of cavernous sinus syndrome. Both sides and genders are equally affected. Bilateral involvement is possible, but rare (in 4-6%). The average age at presentation in one large study was 39, in another 55 years. In the majority of cases, CSS is caused by tumors.

Genetic inheritance

No genetic background.

Pathophysiology

The cavernous sinuses are paired extradural venous plexuses surrounded by dura and the pituitary gland in the center. These cavernous sinuses contain the cranial nerves III (oculomotor nerve), IV (trochlear nerve), V1 (ophthalmic nerve), V2 (maxillary nerve) and VI (abducens nerve), the internal carotid arteries, and their sympathetic plexus. Tumors (primary or secondary), carotid artery aneurysms, carotid-cavernous fistulas, and inflammatory processes are the main causes of this disorder and symptoms are predominantly the result of nerve compression and/or expanding processes in the cavernous sinus.

Diagnosis

The syndrome can be diagnosed clinically. CSS is defined as involvement of two or more cranial nerves or oculosympathetic fibers on the same side, or involvement of only cranial nerve in combination with a radiologically confirmed cavernous sinus lesion. Funduscopy may show optic disc edema and retinal hemorrhages; however, diagnostic imaging and laboratory studies are required to establish the nature of the lesion.

Clinical aspects

On examination, patients may present with ophthalmoplegia, orbital congestion, and proptosis. Cranial nerve palsies may be isolated (in 42%) or comprise a combination of third, fourth, fifth, and sixth cranial nerve palsies (two cranial nerves involved in 28%, three in 20%, and four in 10%). Diplopia is the most common presenting symptom (in approximately 66% of patients), while complete ophthalmoplegia is present in 17-27%. The ophthalmoplegia may be associated with periocular pain (in about 35%) that can be severe, paresthesias in the ophthalmic branch of the trigeminal nerve, and a decreased or absent corneal reflex. Conjunctival congestion and arterialization of the conjunctival veins may be present. Ocular pulsations suggest a vascular fistula. The pupil ...

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