carver no us sinus syndrome

8/8/2019 Carver No Us Sinus Syndrome

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Carvernous sinus syndrome


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BackgroundCavernous sinus syndrome is defined by its resultant

signs and symptoms: ophthalmoplegia, chemosis,

proptosis, Horner syndrome, or trigeminal sensory

loss. Infectious or noninfectious inflammatory,vascular, traumatic, and neoplastic processes are the

principal causes.

Examples of specific entities that may result in

cavernous sinus syndrome are myriad and includecarotid artery aneurysms, carotid-cavernous fistulas

(C-C fistulas), tumors, and Tolosa-Hunt syndrome, to

name the most frequently discussed.


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Pathophysiology

The cavernous sinuses are paired, venous structures

located on either side of the sella turcica. They

receive venous tributaries from the superior and

inferior orbital veins and drain into the superior andinferior petrosal sinuses. The cavernous sinus

contains the carotid artery, its sympathetic plexus,

and the oculomotor nerves (third, fourth, and sixth

cranial nerves). In addition, the ophthalmic branchand occasionally the maxillary branch of the fifth

nerve traverse the cavernous sinus. The nerves pass

through the wall of the sinus while the carotid artery

passes through the sinus itself.


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Cavernous sinus tumors

Cavernous sinus tumors are the most common cause

of cavernous sinus syndrome. Tumors may be

primary or may arise from either local spread or as

metastases. Examples of primary tumors includemeningiomas or neurofibromas. Examples of locally

spreading tumors are nasopharyngeal carcinoma or

pituitary tumors. Metastatic lesions are most often

from the breast, prostate, or lung. Radiotherapy mayoffer transient relief, particularly in nasopharyngeal

cancer. Lateral extension of pituitary tumors may be

treated with surgical resection and dopamine

agonists in the case of prolactinoma. Total resection


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Cavernous sinus aneurysms

Unlike intracranial aneurysms in other anatomic

locations, carotid-cavernous aneurysms do not

involve a major risk of subarachnoid hemorrhage.

However, their rupture can result in direct C-Cfistulas, which may lead to cerebral hemorrhage.

These aneurysms, which are more frequent in the

elderly population, present with an indolent

ophthalmoplegia. Although some patients sufferminor disability and do not require treatment,

endovascular occlusion of these lesions is often

successful and may be attempted in selected

patients.


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Carotid-cavernous fistulas

C-C fistulas are of 2 types: direct and indirect. Direct

fistulas occur if the carotid artery and cavernous

sinus are in continuity. They manifest with abrupt

onset of proptosis, chemosis, visual loss, andophthalmoplegia. Indirect fistulas occur with

communication between the cavernous sinus and the

branches of the internal carotid artery, external

carotid artery, or both.They have a more insidious presentation than direct

fistulas, often with spontaneous resolution. Trauma or

aneurysm rupture is a common cause of carotid-

cavernous fistulas. Interventional radiologists can


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Frequency

In the US, approximately 5% of ophthalmoplegias are

secondary to involvement of cranial nerves in the

cavernous sinuses. This is probably true

worldwide.Cavernous sinus aneurysms represent 5%of all intracranial aneurysms.


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Mortality/

Morbidity

Most of the lesions affecting the cavernous sinuses are

treatable.

Metastatic cancer is a frequent cause of cavernous

sinus syndromes, and the prognosis depends on thespecific tumor type.

Cavernous sinus septic thrombophlebitis mortality has

decreased from 100% to 20% with the

implementation of improvement in diagnosis and

therapeutics.

Cavernous sinus aneurysms and C-C fistulas can be

treated successfully by endovascular techniques.


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Clinical

The signs and symptoms frequently found in patients

with cavernous sinus lesions include visual loss,

proptosis, ocular and conjunctival congestion,

elevation of ocular pressure, ophthalmoplegia, andpain. Various combinations of these symptoms may

occur, which generally are unilateral, but may be

bilateral with neoplastic processes.

Symptoms may be acute or slowly progressive. Primarytumors are the most frequent neoplasm responsible

for a cavernous sinus syndrome.


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Cavernous sinus tumorsAcute or slowly progressive ophthalmoplegia is the

dominant presentation, with diplopia being the

most common symptom.

At times, painful diplopia is present.Usually the patient has a preceding history of cancer.

Occasionally, cavernous sinus syndrome is the first

manifestation of a systemic neoplasm.

Exophthalmos can be seen.

If the tumor is a pituitary adenoma, endocrine

symptoms and visual field deficits may be present.


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Carotid-cavernous aneurysmsPatients frequently are elderly and present with

subacute or chronic ophthalmoplegia.

Rarely, they may have pain similar to that of

trigeminal neuralgia.Spontaneous rupture of a carotid-cavernous

aneurysm leads to an abrupt onset of a direct C-C

fistula. This results in acute onset of massive

exophthalmos with orbital, ocular, and conjunctival

chemosis, binocular diplopia, and visual loss.


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Two types of C-C fistulasDirect fistulas present with prominent acute

symptoms.

Conversely, indirect fistulas are characterized by

mild proptosis, chronic diplopia, drooping of the lid,a red eye, arterialization of the conjunctival

vessels, and visual loss. The patient may report

subjective "noises" in his or her head.


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Cavernous sinus thrombosisThis is infrequent in the postantibiotic era.

It may occur as a complication of infection in the

ethmoid, sphenoid, or frontal sinuses or from

midfacial, dental, or orbital infections.Patients may present with sepsis or metastatic

spread of septic emboli, most commonly

occurring in the lung. This presentation may

appear as acute respiratory distress syndrome

(ARDS).

Retrobulbar pain, drooping of the upper eyelid, and

diplopia may be the first symptoms indicating thelesion's extension to the cavernous sinus.


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Miscellaneous inflammatory lesionsThese may involve the cavernous sinuses or the

walls of the sinus.

Herpes zoster in its acute or chronic stage rarely

causes pain, diplopia, and a droopy eyelid inaddition to the typical zoster blisters. In the chronic

stage, a scar from the acute lesion usually is

found.

An idiopathic inflammation of the walls of the

cavernous sinuses is referred to as Tolosa-Hunt

syndrome.

Sarcoid or Wegener granulomatosis may alsoredis ose to cavernous sinus s ndrome.


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Physical

Cavernous sinus lesions are characterized by thefollowing signs:

Unilateral and isolated third, fourth, or sixth cranial

nerve palsyCombination patterns of ophthalmoplegia

Painful ophthalmoplegia

Proptosis (pulsating exophthalmos suggests a direct

C-C fistula)Ocular and cranial bruits

Conjunctival congestion; arterialization ofconjunctival veins

Ocular hypertension


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Anesthesia in the ophthalmic division of thetrigeminal nerve (V1) and/or decreased or absentcorneal reflex and possibly anesthesia in themaxillary or V2 branch

Pupil in midposition and nonreactive if bothsympathetics and parasympathetics from the thirdnerve are affected


Metastatic lesions - Isolated or combinedophthalmoplegia, painful ophthalmoplegia,anesthesia in the ophthalmic nerve

Pituitary tumors - Isolated or combinedophthalmoplegia (lateral extension); endocrine

signs such as acromegaly, galactorrhea, and


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Isolated or combined ophthalmoplegia


Decreased pain sensation in the V1 ophthalmic

divisionCarotid-cavernous fistulas

Direct - Unilateral massive proptosis, pulsatingexophthalmos, lid congestion, conjunctival

chemosis, orbital congestion, ocular hypertension,visual loss, optic neuropathy, optic disc edema,retinal hemorrhages, retinal venous congestion,and loud ocular and cranial bruit

Indirect -S

imilar signs and symptoms of lesserseverit occasionall isolated o hthalmo le ia


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Cavernous sinus thrombosisGenerally speaking, primary infectious process

involving paranasal sinuses and/or orbital cellulitis

In addition to local and systemic signs of infection,

the following may be seen:Isolated or combined ophthalmoplegia


Orbital congestion, lid chemosis, proptosisVisual loss, optic disc edema (unilateral)

Signs of meningeal irritation


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Miscellaneous cavernous sinus lesionsTolosa-Hunt syndrome - Isolated or combined,

painful ophthalmoplegia

Herpes zoster - Acute zoster ophthalmicus, typical

skin lesion, and keratitisSarcoidosis - Systemic signs, uveitis,

ophthalmoplegia, facial diplegia


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Causes

Metastatic tumors

Breast

Prostate

Lung

Localized spread of tumor

Nasopharyngeal

Pituitary


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Primary intracranial tumorsMeningiomas

Neurofibromas

Chondromas (less common)

Trauma (including postsurgical)

Carotid-cavernous aneurysms


Cavernous sinus thrombosis


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Miscellaneous inflammatory syndromesHerpes zoster

Tolosa-Hunt syndrome

Sarcoidosis


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Differentials Aneurysms involving posteriorcommunicating artery with painful ophthalmoplegia

Orbital pseudotumor

Basilar artery aneurysm

Paranasal sinus lesions (particularly in sphenoidsinus)

Causes of cisternal third, fourth, and sixth nerve

palsies, including basilar meningitides

Thyroid eye diseaseIntra-axial brainstem lesions associated with isolated

ophthalmoplegia

Tumors and other lesions in the middle cranial fossa

Lesions involving orbital apex, especially in and


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Diagnostic Workup

Patients in whom cavernous sinus lesions aresuspected should undergo thin-section multiplanarimaging studies of the orbit and the sellar/parasellarregion. Precontrast and postcontrast scans are

advisable. CT scan offers better visualization of boneand calcium. However, MRI provides better detail ofall soft tissues contained in the sinuses, the expectedsignal void of the normal carotid artery, and its

relation to the surrounding structures.Orbital views are necessary to exclude a disease

process primarily involving the cavernous sinuseswith concomitant compromise of the orbital apex.

Conversely, primary orbit and paranasal sinus


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In the case of metastatic tumors, diagnosis of the

primary neoplasm generally precedes the cavernous

sinus syndrome.

A lumbar puncture with cytologic examination may behelpful.

If a primary neoplasm of the nasopharynx is suspected,

a biopsy may be needed.

In the case of pituitary tumors with lateral extension,

tumor resection may be necessary.

Rarely, a biopsy of the cavernous sinus tumor is

needed for diagnosis.


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As MRI and/or magnetic resonance angiography (MRA)

are often specific, cerebral angiography generally is

not required to make a diagnosis.

Perform angiography if balloon occlusion of theaneurysm is planned.


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The dramatic clinical presentation and MRI and/or MRA

of direct fistulas leave little doubt regarding the

diagnosis.

By contrast, indirect fistulas, particularly those drainingin the petrosal sinuses, are associated with subtle

findings and possibly a normal MRI and/or MRA.

Cerebral angiography is the only way to arrive at the

correct diagnosis.

Perform angiography to stage the fistula and document

the anterior and posterior drainage routes.


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C

avernous sinus thrombosisImaging of the orbit and/or nasal sinuses is helpful in

the search for a septic focus.

An aseptic thrombosis may be associated with

conditions such as hypercoagulable states andlymphoproliferative disorders.

A hematologic workup is indicated in these patients.


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Miscellaneous inflammatory syndromes

Investigate additional systemic inflammatory and

granulomatous processes after other common

causes have been excluded. These disorders can be

screened by studies such as laboratory tests andchest x-ray.

Tolosa-Hunt syndrome (an uncommon, idiopathic,

inflammatory cavernous sinus syndrome) may share

a close etiologic link with orbital pseudotumor.A nonspecific fibrotic inflammatory reaction and

rarely a granulomatous inflammation can occur,

but biopsy is rarely used to establish the diagnosis.

A ositive res onse to steroids is considered


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Biopsy of the cavernous sinuses entails a craniotomy,which is associated with morbidity. Restrict acraniotomy to patients with a documented,progressive cavernous sinus syndrome.

Treatment Cavernous sinus tumors

Metastatic lesionsRadiotherapy may offer transient improvement.

Nasopharyngeal carcinomas may be veryradiosensitive, with a prolonged remissionfollowing treatment.

Pituitary tumors

Prolactinomas may improve with oral dopamineagonists, or they may require resection.

Gamma knife ma be an ad unctive treatment for


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Cavernous sinus meningiomas

These lesions represent a major challenge for

surgical resection, primarily because of poor

accessibility and the frequent encasement of the

cavernous carotid artery.Since these tumors are slow growing and difficult to

resect, elderly patients or those with minor

symptoms probably should be observed

expectantly without specific treatment.Radiotherapy may be offered to some patients, and

some show improvement of cranial nerve function

after gamma knife treatment. Partial resection may

be attempted in others with disabling symptoms


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Treat cavernous sinus aneurysms by endovascularballoon occlusion.

Carotid-cavernous fistulas Treatment ideallyconsists of endovascular obliteration of the fistulawith coils, although some cavernous sinus dural AVfistulas may be observationally managed.

Access to the fistula may be transarterial; however, thetransvenous approach has become the mainstay of

treatment, in some cases a combinedsurgical/endovascular approach can be used withsurgical exposure of the superior ophthalmicvein followed by fistula embolization. Several venous

approaches have been used, including the safest


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Percutaneous transorbital access to the cavernous

sinus followed by embolization.

Small indirect C-C fistulas may occlude either

spontaneously or following diagnostic angiography. If

the clinical signs are mild, consider carefulmonitoring.

If intraocular pressure is elevated, antiglaucoma agents

may be required.


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Cavernous sinus thrombosis

High-dose antibiotic therapy should be directed

against the most common pathogens, such as S

aureus and S pneumoniae, as well as gram-negative

rods and anaerobes.Anticoagulation in septic cavernous sinus thrombosis is

controversial, but may hasten the rate of recovery.

Drainage of any primary site of infection (eg, abscess,

sinusitis) is advised.Corticosteroids are not recommended.

Anticoagulation also may be helpful in aseptic patients.


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Miscellaneous inflammatory syndromes

Inflammatory cavernous sinus syndromes may

respond to treatment of the specific systemic

inflammation or vasculitic etiology.

Tolosa-Hunt syndrome responds well to a 3- to 6-monthcourse ofhigh-dose steroid therapy that can be

tapered slowly thereafter.

Keywords

parasellar lesions, cavernous sinus, tumors, carotidartery aneurysms, carotid-cavernous fistulas, C-C

fistulas, cavernous sinus syndrome, ophthalmoplegia,

chemosis, proptosis, Horner syndrome, Horner's

syndrome, trigeminal sensory loss, cavernous sinus


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carver no us sinus syndrome

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