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.
Foix Syndrome; Jefferson Syndrome; Foix-Jefferson
Approximately 5% of all ophthalmoplegias are the
result of cavernous sinus syndrome.
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.
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.
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.
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
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.
In the presence of increased
intracranial pressure, drugs that increase cerebral blood flow should be
avoided or used only at low concentrations/doses.
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
Keane JR: Cavernous sinus syndrome: Analysis of 151 cases. Arch Neurol
Van Overbeeke JJ, Jansen ...