Cst

FIRST CONSULT

Cavernous sinus thrombosisRevised: September 23, 2011Copyright Elsevier BV. All rights reserved.

Key points

Cavernous sinus thrombosis is an uncommon but life-threatening condition in which a clot

forms in the cavernous sinus, a cavity through which blood from the cranial area flows back to

the heart

Typically seen in conjunction with proptosis, ptosis, chemosis, and cranial nerve palsy that

begins in one eye and progresses to the other

Usually results from an infection in the face or paranasal sinuses

Adverse effects can include papilledema, fever, and headache and often palsies of some cranial

nerves

Both sexes are at equal risk, but a strong correlation with oral contraceptive use increases the

incidence among women

Magnetic resonance imaging (MRI), computerized axial tomography (CT), and a complete

blood count (CBC) are tests used to diagnose the condition

Treatment for cavernous sinus thrombosis includes broad-spectrum intravenous antibiotic

medications and surgery

Since the advent of antibiotic medications, the mortality rate has fallen to between 20% and

30%, from between 80% and 100%

The timely and comprehensive treatment of infections of the face and paranasal sinuses will

help prevent cavernous sinus thrombosis

If condition is suspected, the patient should be transferred immediately to a hospital for

intravenous antibiotic therapy

Background

Description

Cavernous sinus thrombosis is an uncommon but life-threatening condition in which a clot

forms in the cavernous sinus

The classic presentation is of proptosis, ptosis, chemosis, and cranial nerve palsy beginning

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in one eye and progressing to the other eye

Most cases follow infection of the sphenoid sinuses, eyelid, orbit, face, upper teeth, or

middle ear

Commonly causes papilledema (80% of cases), fever, and headache and often palsies of

cranial nerves III, IV, V, and VI

Treated with broad-spectrum intravenous antibiotic medications: nafcillin, cefotaxime,

ceftriaxone, and metronidazole

Mortality rate is approximately 30%

Epidemiology

Incidence and prevalence:

Cavernous sinus thrombosis is rare. The average physician is unlikely to encounter a single

case in an entire career

The true incidence is unknown. An incidence of less than 0.01/1,000/y has been estimated

in Saudi Arabia

Gender:

Both sexes are at equal risk, but a strong correlation with oral contraceptive use increases

the incidence among women

Genetics:

Congenital coagulation disorders, factor V Leiden mutation, and factor II mutation increase

the relative risk of venous thromboembolism generally

Causes and risk factors

Causes

Common causes:

Sphenoid sinusitis

Otitis media

Orbital cellulitis

Maxillary sinusitis

Dental infection

Facial cellulitis

Pharyngitis


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Tonsillitis

Fungal infections (especially Mucor and Aspergillus species)

Rare causes:

Head injury

Tumors

Leukemias

Coagulopathies

Contributory or predisposing factors

Diabetes mellitus increases the risk for infection, as well as the risk of septic cerebral sinus

thrombosis

Combined oral contraceptive use increases the risk for venous thrombosis generally by

between 2- and 4-fold

Prothrombotic genetic mutations: factor V Leiden and factor II mutation. When combined

with oral contraceptives, these mutations increase the relative risk of cerebral sinus

thrombosis 10- to 20-fold

Pregnancy

Malignancy

Smoking

Illicit drug use ( ie, intravenous injection)

Associated disorders

Venous thrombosis at another site: Noncerebral thrombotic events occurred in 14% of a series of

patients with cerebral venous thrombosis observed for an average of 63 months.

Screening

Summary approach

Screening for thrombophilia in the general population, particularly in women starting a

combined oral contraceptive, is controversial.

Population at risk

Combined oral contraceptive use increases the risk for venous thrombosis generally by

between 2- and 4-fold

Patients with the prothrombotic genetic mutation factor V Leiden and factor II mutation

who are taking oral contraceptives have a 10- to 20-fold increased relative risk for cerebral


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sinus thrombosis

Primary prevention

Summary approach

The timely and comprehensive treatment of infections of the face and paranasal sinuses will help

prevent cavernous sinus thrombosis. These usually occur in the week or 2 before the onset of the

thrombosis.

Population at risk

Hypercoagulability: Thrombophilia was found in 20% of patients with cavernous sinus

thrombosis in 1 series ( ie , patients with the prothrombotic genetic mutation factor V

Leiden and factor II mutation)

Intravenous drug misuse: There was a case report of cavernous thrombosis following illegal

injection of heroin into the carotid artery

Those using oral contraceptives

Preventive measures

The avoidance of injection of illegal drugs into the carotid artery will reduce the risk of

cavernous sinus thrombosis

A family history of recurrent thrombosis may prompt the search for thrombophilia, which

in the context of oral contraceptive use has a positive correlation with cavernous sinus

thrombosis

Diagnosis

Summary approach

The combination of ptosis, proptosis, chemosis, and ophthalmoplegia in an acutely unwell patient

with headache are the hallmarks of cavernous sinus thrombosis. The diagnosis is a clinical one as

far as the general physician is concerned. The patient should be admitted to the hospital without

delay.

Clinical presentation

Symptoms

Headache is unilateral or retro-orbital and is present at initial presentation in 80% of cases

Decreased visual acuity and diplopia

Sensory deficits of the ophthalmic and maxillary branches of the fifth cranial nerve with

numbness and/or tingling in the forehead, periorbital, and midface regions

Signs

Seventy-five percent of patients have eye complaints at presentation:


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Periorbital edema, initially unilateral but spreading quickly to the contralateral eye

Ptosis

Proptosis

Ophthalmoplegia, especially sixth nerve palsy

Chemosis

Other findings may include the following:

Fever in nearly all patients

Tachycardia

Facial cellulitis

Papilledema in 70% to 80%

Decreased visual acuity in 20%

Nuchal rigidity in 30%

Seizures in 10%

Examination

Assess the patient's general state of health. In many cases of cavernous sinus thrombosis,

the patient is acutely unwell at presentation, with fever, tachycardia, signs of sepsis,

drowsiness, or even convulsions or coma

Examine the patient's eyes. Signs of proptosis, ptosis, and chemosis are often present,

usually in one eye at presentation but rapidly progressing to the contralateral eye. At the

back of the eyes, ophthalmoscopy may reveal papilledema

Examine the patient's face. Evidence of facial edema, eyelid edema, or the facial swelling

and redness of acute sinusitis may be present

Assess whether the patient can look upward, downward, and to the left and right.

Ophthalmoplegia is commonly due to involvement of the third, fourth, and sixth cranial

nerves as they cross the cavernous sinus

Test the patient's vision. Visual acuity is often reduced, sometimes to complete blindness in

one eye

Test the patient's sense of touch on the face. Sensation of the skin and cornea are often

reduced in the distribution of the ophthalmic or maxillary branches of the facial nerve

Assess the patient's ability to flex his or her neck. Nuchal rigidity often accompanies the

headache

Questions to ask


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Presenting condition:

Do you have a headache? More than 80% of patients with cavernous sinus thrombosis have

a headache at presentation. It is usually unilateral, retro-orbital, or along the ophthalmic or

maxillary branches of the trigeminal nerve

Can you see normally? Most patients present with evidence of ophthalmoplegia, which

causes double vision. Retro-orbital compression of the retinal vein causes reduced visual

acuity or blindness

Do you feel unwell? Fever, tachycardia, and sepsis are common presenting features

Do you have any numbness of the face? Involvement of the facial nerve is not uncommon

Contributory or predisposing factors:

Have you recently had any sinus infection or other infection of the face? This is the single

most likely predisposing factor for cavernous sinus thrombosis

Do you have diabetes mellitus? This increases the risk of infection as well as the risk of

septic cerebral sinus thrombosis

Are you taking a combined oral contraceptive? Use of this class of drugs increases the risk

of venous thrombosis generally by between 2- and 4-fold

Do you have any prothrombotic genetic mutations? For example, factor V Leiden and

factor II mutation? When combined with oral contraceptives, these mutations increase the

relative risk of cerebral sinus thrombosis 10- to 20-fold

Are you pregnant or have you recently been pregnant? This increases the thrombotic risk

generally

Do you have any malignancy? This also increases the risk of thrombosis generally

Are you on any immune-suppressant drugs? Corticosteroids and chemotherapeutic agents

predispose to cavernous sinus thrombosis

Diagnostic testing

Magnetic resonance imaging (MRI) (481216)of the brain with and without contrast. This test isthe cornerstone of diagnosis, and can be combined with angiography to demonstrate the

presence of clot in the cavernous sinus

Cerebral angiography (1207007)( ie , the venous phase) may be a useful adjunct to MRI and CTmodalities

Complete blood count (481192). This test will show a leukocytosis in more than three-quartersof cases. Although it can be performed by the general physician, it should not be allowed to

delay the urgent transfer of the patient to hospital

Coagulation panel (1207006)in patients with otherwise unexplained cavernous sinus


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thrombosis

Computerized axial tomography (CT) (481409). In the absence of MRI facilities, this will usuallydemonstrate the anatomy of the cavernous sinus and any surrounding sinusitis

Magnetic resonance imaging

Description

A sensitive, noninvasive method of imaging the internal structures of the head by placing

the patient inside a rotating magnetic field. Can be combined with angiography to

demonstrate lack of blood flow in the cavernous sinus

Normal results

No intracranial abnormality is seen

Comments

Movements, arterial pulsation, and foreign bodies might produce artifacts and interfere

with the interpretation

Not available for some patients with metal prostheses/pacemakers

Claustrophobia is a problem with some patients

MRI findings may be subtleif cavernous sinus thrombosis is suspected, this must be

specifically asked about

Cerebral angiography

Description

Evaluates the blood vessels that supply the brain ( ie , the carotid arteries and the

vertebral arteries)

A catheter is placed through the femoral artery in the thigh and maneuvered to the head

under radiologic guidance. When the catheter is in place, dye is injected into the

bloodstream while radiographs are taken to allow clear visualization of the blood vessels.

Radiographs are taken at several time points and from several angles to visualize the

arteries and the venous system in the head

Normal results


Comments

MR angiography and CT angiography are used in some centers and may eventually

replace conventional angiography

CT and MR angiography may miss small lesions


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Keep in mind the possibility of a false-positive result

Risk of morbidity and mortality

Complete blood count

Description

Venous blood sample

Normal ranges

Leukocyte count: 4,500 to 11,000/L

Erythrocyte count: 3.9 to 5.5 106/L

Hemoglobin: 14.0 to 17.5 g/dL

Hematocrit: 41% to 50%

Platelet count: 150 to 350 103/L

Comments

Other foci of bacterial infection, such as orbital cellulitis and sinusitis, may alter results

Test results will confirm a leukocytosis in most cases of septic cavernous sinus

thrombosis

There will be a delay in obtaining the result. In unwell patients, this should not be

allowed to delay the urgent transfer to hospital

Coagulation studies

Description

Includes prothrombin time (PT), partial thromboplastin time (PTT), lupus anticoagulant

screen, anticardiolipin antibodies (IgG, IgA, and IgM), an activated protein C resistance

(followed by a confirmatory Leiden V mutation test if abnormal), a circulating

anticoagulant screen, and a prothrombin variant mutation test

Normal results

PT: 12 to 14 seconds

PTT: 18 to 28 seconds

Lupus anticoagulation screen: consult local laboratory standards

Anticardiolipin antibodies: consult local laboratory standards

Activated protein C resistance: consult local laboratory standards

Circulating anticoagulant screen: consult local laboratory standards


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Prothrombin variant mutation test: consult local laboratory standards

Comments

Use of any anticoagulant medications such as heparin, heparinoids, warfarin, dabigatran,

or oral antifactor Xa inhibitors must be reported to the laboratory at the time of

collection

Not justified in cases when there is a clear underlying cause ( eg , adjacent sinus infection

or neoplasm)

Computerized axial tomography

Description

A detailed three-dimensional radiograph imaging technique to demonstrate the internal

bony structures of the head

Normal results


Comments

Movements, arterial pulsation, and foreign bodies might produce artifacts and interfere

with the interpretation of test results

Differential diagnosis

Orbital cellulitis

Orbital cellulitis is an acute infection of the tissues surrounding the eye (eyelids, eyebrow,and cheek)

It is usually unilateral and features lid edema, rhinorrhea, orbital pain, tenderness, dark

red discoloration of eyelids, and fever

There is no involvement of the ophthalmic division of the trigeminal nerve

Ophthalmoplegic migraine

Ophthalmoplegic migraine is a cranial neuropathy, thought to be a recurrent

demyelinating process, that affects cranial nerves III (most common), IV, or VI

It is unilateral and recurrent. Features include retro-ocular headache, tearing, no fever, a

prodromal phase, temporary ophthalmoplegia

Carotid cavernous sinus fistula

Carotid cavernous sinus fistula is an abnormal communication between the internal or

external carotid arteries and the cavernous sinus

Features include ocular bruit, apyrexial, headache, ophthalmoplegia, proptosis, severe

conjunctival injection and edema


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Mucormycosis

Mucormycosis is infection in the paranasal and orbital structures caused by Zygomycetes

fungi

Features include fever; facial pain; diplopia; proptosis; and necrotic ulceration of the

palate, pharynx, or nasal septum

Occurs in diabetic or immunocompromised patients

Tolosa-Hunt syndrome

Tolosa-Hunt syndrome is an acute idiopathic inflammatory process of the superior

orbital fissure or the anterior cavernous sinus

Features include painful ophthalmoplegia without proptosis, no fever

MRI might show some abnormal signal in the superior orbital fissure or anterior

cavernous sinus

Responds excellently to steroids

Orbital pseudotumor

Orbital pseudotumor is an idiopathic inflammatory process that involves the orbit

(enlargement of the extraocular muscles and other contents of the orbit)

Usually unilateral with slight proptosis with painful ophthalmoplegia but the patient

does not look septic

There is no fever and no involvement of ophthalmic division of the trigeminal nerve

CT scan of the orbit shows enlargement of the orbital contents, as in thyroid

ophthalmopathy

Responds to steroids

Carotid artery aneurysm or dissection in the cavernous portion

Carotid artery aneurysm or dissection in the cavernous portion are clinically almost

impossible to differentiate

No signs of sepsis and none of them are preceded by sinus infection

Brain imaging is needed to make the diagnosis

Neoplasm

Usually the symptoms and signs of neoplasms are not limited to the cavernous sinus

Brain MRI might be sufficient to make the diagnosis

Pseudotumor cerebri

Pseudotumor cerebri features headache associated with papilledema, visual


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obscurations, and/or intermittent horizontal diplopia

Fever is not expected

Confirmed by normal imaging studies and high cerebrospinal fluid pressure on lumbar

puncture

Temporal arteritis

Temporal arteritis (or giant cell arteritis) is a vasculitis of the large and medium-sized

arteries, predominantly affecting arteries of the carotid system

Temporal headache in middle age or elderly patients

Elevated ESR in the vast majority of patients

Temporal artery is thickened and tender

Papilledema is not present

Fever may exist

Ischemic optic neuropathy but no cranial nerve III, IV, V, or VI palsies

Consultation

In the early stages of cavernous sinus thrombosis, the only feature may be a headache. If

there is a history of facial or sphenoid sinus infection in the previous week, and the

headache is unilateral, retro-orbital, or along the ophthalmic or maxillary branches of the

trigeminal nerve, give serious consideration to the possibility of cavernous sinus

thrombosis. Referral to a specialist or the emergency department is indicated. The

development of periorbital edema, facial cellulitis, proptosis, or ptosis should indicate the

need for urgent care

Conditions such as orbital cellulitis, mucormycosis, and idiopathic intracranial

hypertension share many of the features of cavernous sinus thrombosis. Admission to a

hospital for diagnostic assessment may be indicated. The urgency of admission should be

dictated by the patient's general condition, but the combination of ophthalmoplegia, fever,

and headache warrants urgent assessment

Referral to specialists in neurology and infectious disease should be considered immediately

Refer for surgical consultation for drainage of infected sinuses

Treatment

Summary approach

Goals:

To prevent morbidity from visual loss, diplopia, seizures, propagation to other adjacent

cerebral venous sinuses and other thrombotic events and the loss of life


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To identify the specific underlying cause of the cavernous sinus thrombosis, especially

causes such as hypercoagulable states, which may predispose to future thromboembolic

events in the patient or in family members if the hypercoagulable state is heritable

Immediate action:

If the diagnosis cannot be confirmed because of the absence of imaging facilities,

intravenous antibiotic therapy can be commenced empirically, as most cases of cavernous

sinus thrombosis are due to infection. The patient must subsequently be transferred to a

facility with further diagnostic capability. If the patient is diabetic, consider empiric

treatment for mucormycosis as well, as its clinical presentation is often identical to that of

cavernous sinus thrombosis and potentially as deadly

Summary of therapies:

Before the advent of antibiotic medications, the mortality rate for cavernous sinus

thrombosis was between 80% and 100%. Even with the use of modern broad-spectrum

intravenous antibiotic therapy, the mortality rate is still between 20% and 30% and

morbidity between 25% and 50%. Some of this improvement in outcome is due to

improvements in the general health of the population and in nursing and other supportive

care, and should be viewed against a fall in mortality for all infectious diseases over the last

50 years

Treatment usually begins with nafcillin or oxacillin given in combination with a third-generation cephalosporin such as ceftriaxone or cefotaxime

Metronidazole intravenously, which covers anaerobic organisms, should be added if dental orsinus infection is suspected

Vancomycin is used for patients with -lactam allergy or when methicillin resistance isanticipated

Heparin is often given, although this is controversial if the underlying cause is infectious

Corticosteroids such as hydrocortisone may be used as an adjunct to antibiotic therapy torelieve inflammation, and they are certainly indicated in pituitary failure

Surgery (481290)may be done by a specialist to drain any infected sinus of mucus or focus ofinfection, either endoscopically or directly

Medications

Nafcillin

Indication

Nafcillin is a semisynthetic penicillin used in the treatment of cavernous sinus thrombosis

Dose information

1.5 g by intravenous infusion over 60 minutes every 4 hours


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Major contraindications

Corn hypersensitivity

Penicillin hypersensitivity

Comments

Use caution in pediatric patients. There are no approved neonatal or pediatric dosage

regimens for intravenous nafcillin

Vein irritation at the intravenous administration site can be a problem with nafcillin.

This is reduced by infusing each dose over 60 minutes

Oxacillin

Indication

Oxacillin is a semisynthetic penicillin used to treat cavernous sinus thrombosis

Dose information

1 g by slow intravenous injection over 10 minutes every 4 hours

Comments

Warning: With intravenous administration, particularly in elderly patients, care should

be taken because of the possibility of thrombophlebitis

Use caution in pediatric patients and neonates

Good coverage of all Gram-positive bacteria, including the penicillinase-producing

staphylococci

Ceftriaxone

Indication

Ceftriaxone is a cephalosporin antibiotic used in the treatment of cavernous sinusthrombosis

First-line treatment for this disorder in combination with nafcillin or oxacillin

Dose information

2 g intravenously every 12 hours


Cephalosporin hypersensitivity

Jaundice

Comments


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Must be administered intravenously; other routes are not adequate for this disorder

Cefotaxime

Indication

Cefotaxime is a cephalosporin antibiotic used to treat cavernous sinus thrombosis

Dose information

1.5 to 2 g by intravenous injection or infusion every 4 hours


Cephalosporin hypersensitivity


Comments

Warning: risk of granulocytopenia and (more rarely) agranulocytosis, particularly if

given over long periods

Vancomycin

Indication

Vancomycin is used for cavernous sinus thrombosis patients with a -lactam allergy orwhen methicillin resistance is anticipated

Dose information

1 g intravenously every 12 hours

Vancomycin blood levels need to be taken daily and monitored. Dose may require

adjustment depending on level



Vancomycin hypersensitivity

Comments

Warning: some patients with intestinal inflammation disorders may have significant

systemic absorption of drug and may be at risk of adverse effects usually associated with

parenteral administration

Warning: Avoid rapid infusion due to risk of anaphylaxis, hypotension, and cardiac

arrest. Administer over a period of not less than 60 minutes. Rotate sites of infusion to

reduce risk of thrombophlebitis. Avoid extravasation


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Use caution in the elderly

Safety and efficacy in pediatric patients have not been established

Metronidazole

Indication

Metronidazole is an antibiotic and antiprotozoal agent used to treat cavernous sinusthrombosis

Dose information

Loading dose: 15 mg/kg intravenously infused over 1 hour (approximately 1 g for a 70-kg

adult)

Maintenance dose: 7.5 mg/kg intravenously infused over 1 hour every 6 hours

(approximately 500 mg for a 70-kg adult). The first maintenance dose should be

instituted 6 hours following the initiation of the loading dose

Comments

Warning: hypersensitivity reactions including urticaria have been reported rarely

Use caution in the elderly

Heparin

Indication

Heparin is an anticoagulant used to treat cavernous sinus thrombosis

Dose information

80 U/kg bolus followed by 18 U/kg/h by intravenous infusion

Dose is adjusted based on partial thromboplastin time results


Bleeding

Heparin hypersensitivity

Thrombocytopenia

Comments

Should not be given to patients who cannot have suitable blood tests at appropriate

intervals

Use caution in patients with diabetes and electrolyte disturbances

Evidence


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A systematic review of 2 randomized, controlled trials, inclusive of 79 patients,

evaluated the effects of anticoagulation in cerebral sinus thrombosis: one compared

high-dose, subcutaneous, low-molecular weight heparin versus a control; the other

compared intravenous, unfractionated heparin versus a control. The reviewers

concluded that anticoagulation treatment appeared safe in cases of cerebral venous

sinus thrombosis and was associated with a potentially important reduction in the risk

of death or dependency. [1] Level of evidence: 2

References

Hydrocortisone

Indication

Hydrocortisone is a corticosteroid that may be used as an adjunct to antibiotic therapy torelieve inflammation and to treat pituitary failure in patients with cavernous sinus

thrombosis

Dose information

100 mg intravenously every 6 hours


Fungal infection

Comments

Corticosteroids may prove helpful in reducing cranial nerve inflammation, secondary

cranial nerve dysfunction, and decreasing orbital edema

Risk of electrolyte disturbances, hypertension, and water retention. Dietary salt

restriction and potassium supplementation may be necessary. All corticosteroids

increase calcium excretion. Use caution in patients with cardiac disorders ( eg,

congestive heart failure, hypertension, recent myocardial infarction)

Non-drug treatments

Surgical drainage

Description

Surgery to drain any infected sinus of mucus, either endoscopically or directly

Indication

To remove focal nidus of infection ( eg, sinus or dental abscess)

Comments

As bleeding may be a complication, surgical drainage is strictly contraindicated in the

presence of therapeutic anticoagulation


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Surgery may be indicated to drain the sinus cavity itself or an adjacent infectious process

predisposing the patient to cavernous sinus thrombosis

Special circumstances

Comorbidities

Coexisting disease:

Diabetes mellitus : Diabetic control is likely to be compromised during treatment forcavernous sinus thrombosis. Hypoglycemia and hyperglycemia are both possible. Close

monitoring of plasma glucose is necessary

Coexisting medication:

Oral anticoagulants: Patients on warfarin are less likely to suffer an episode of cavernous

sinus thrombosis, but if they do, then careful monitoring of clotting times will be

necessary if heparin is used

Special patient groups:

Allergic patients: A previous anaphylactic reaction to any of the broad-spectrum

antibiotic medications used will require a careful search for a suitable alternative drug.

Lesser degrees of allergy may be overlooked

Pregnancy: Cavernous sinus thrombosis is more common in pregnancy. The condition is

life-threatening, and intravenous broad-spectrum antibiotic medications may save the

lives of both the mother and fetus

Terminal care: Cavernous sinus thrombosis is more common in patients with

malignancy. The mortality from cavernous sinus thrombosis without antibiotic therapy is

between 80% and 100%. The patient may decide not to accept intravenous antibiotic

therapy, in which case the priority is to maintain comfort and dignity throughout the

terminal events

Patient satisfaction/lifestyle priorities

Most patients will gratefully accept transfer to a hospital for intravenous therapy. One feature

of the acute toxic state may be that the patient is confused. Reluctance to accept treatment may

be a sign of this.

Consultation

All patients with suspected cavernous sinus thrombosis should be admitted to the hospital

urgently for intravenous broad-spectrum antibiotic treatment

There is no place for management with oral antibiotic medications or nonantibiotic therapy

alone. The mortality for cavernous sinus thrombosis before the antibiotic era was between

80% and 100%

If admission to hospital is impossible or necessarily delayed, intravenous broad-spectrum

antibiotic treatment should be started as soon as possible


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Follow-up

During the acute phase of the condition, patients are treated in the hospital and their progress is

monitored closely. If the acute episode settles, then outpatient follow-up is important because there

is a risk of complications, such as seizures, focal neurologic deficits, visual problems, and future

thrombotic events.

Plan for review

Acute phase in hospital: regular monitoring of temperature, blood pressure, pulse, neurologic

status, hydration, urine output, and electrolyte balance

Outpatient follow-up: full neurologic examination 4 weeks after hospital discharge, especially

to detect cranial nerve damage and reduced visual acuity, and to ask about seizure activity,

symptoms of other thromboses, and return to normal daily living

Secondary prevention

Long-term oral anticoagulation may be indicated in patients with thrombophilia after

cavernous sinus thrombosis or after recurrent thrombosis, but the evidence for this is lacking

at present

The use of anticonvulsant agents as prophylaxis after cavernous sinus thrombosis is not

supported because of the low risk of seizures in patients who do not experience a seizure

acutely

Prognosis

The overall mortality in this condition is between 20% and 30%

The morbidity is between 25% and 50%

Progression of disease

Therapeutic failure:

The first-choice antibiotic medicationsnafcillin (or oxacillin), cefotaxime, and

metronidazoleare used on an empiric basis. If bacterial culture material can be obtained

from sinus washings, blood culture, or lumbar puncture, this may guide the choice of antibiotic

agent

Recurrence:

In one series of 77 patients, nine (11.7%) suffered a further cerebral sinus thrombosis,

including all but one in the first year. In a series of 40 patients with cerebral venous

thrombosis, 15% to 20% had abnormal clotting factors, the majority being factor V Leiden.

Therefore, in patients with no obvious cause for their cerebral venous thrombosis, long-term

oral anticoagulation may be indicated in those with positive results on screening for

thrombophilia. There is no direct evidence for this approach, which remains controversial


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Deterioration:

With a mortality rate of about 20%, deterioration is not uncommon. General supportive

measures in intensive care may be required, with attention to hydration, temperature control,

nutritional status, respiratory support, circulatory support, and skin care. The prevention of

seizures may be necessary. Intensive nursing care is essential in the comatose patient

Terminal illness:

Pain controlconsider intravenous opiates and nonsteroidal anti-inflammatory medications

Vomitingantiemetic agents given in conjunction with analgesic medications

Anxietyanxiolytic medications such as diazepam or midazolam

Respiratory distressopiates

Seizuresanticonvulsant medications given rectally or intravenously if acute need, and oral

maintenance for chronic treatment

Clinical complications

Septic cavernous sinus thrombosis has a high morbidity, with less than 40% of patients

recovering without complications

The risk of death is between 20% to 30%

Oculomotor palsies, blindness, and hemiparesis are the most common sequelae

Recurrent seizures are seen in 15% of patients who have seizures acutely

A second cerebral venous thrombosis is likely in 11.7% of patients, mostly in the first year

Noncerebral thromboses occur in 14.3% of patients

All thromboses occur in 20% of patients

Rare complications include hypopituitarism and arteriovenous fistula

Patient education

With regard to future medical considerations, having suffered a cavernous sinus thrombosis

puts the patient in the same category as the patient with a deep vein thrombosis and the

following contraindications and cautions apply:

The combined oral contraceptive pill is absolutely contraindicated

Use caution with estrogen replacement therapy

Use caution with surgical procedures and long-haul flights

Use caution with future pregnancies


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There is also a small excess risk of developing seizures in the future, although this is not great

enough to warrant starting prophylactic anticonvulsants

Admission to hospital should be strongly recommended. If necessary, the patient should

be informed that the mortality rate without intravenous antibiotic therapy is close to

100%. With correct treatment the mortality rate is reduced to approximately 30%

Some patients do not like taking antibiotic medications for fear of becoming immune to

them. This misconception should be dealt with by conveying the mortality rate

information

Online information for patients

National Health Service: Cavernous sinus thrombosis (http://www.nhs.uk/conditions/Cavernous-sinus-thrombosis/Pages/Introduction.aspx)

Resources

Summary of evidence

Evidence

A systematic review of 2 randomized, controlled trials, inclusive of 79 patients, evaluated

the effects of anticoagulation in cerebral sinus thrombosis: one compared high-dose,

subcutaneous, low-molecular weight heparin versus a control; the other compared

intravenous, unfractionated heparin versus a control. The reviewers concluded that

anticoagulation treatment appeared safe in cases of cerebral venous sinus thrombosis

and was associated with a potentially important reduction in the risk of death or

dependency. [1] Level of evidence: 2

References

References

Evidence references

1. Coutinho J, de Bruijn SF, Deveber G, Stam J. Anticoagulation for cerebral sinus

thrombosis. Cochrane Database Syst Rev. 2011:CD002005

View In Article (refInSitu18196)

Guidelines

The European Federation of Neurological Societies (http://www.efns.org/)has produced the following:

Einhaupl K, Bousser MG, de Bruijn SF, et al. EFNS guideline on the treatment of cerebralvenous and sinus thrombosis (http://www.efns.org/fileadmin/user_upload/guidline_papers/EFNS_guideline_2006_treatment_of_cerebral_venous_and_sinus_thrombosis.pdf). Eur J Neurol.2006;13:553-9

Further reading

Laupland KB. Vascular and parameningeal infections of the head and neck. Infect Dis

Clin North Am. 2007;21:577-90


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Wald ER. Periorbital and orbital infections. Infect Dis Clin North Am. 2007;21:393-408

Roscoe DL, Hoang L. Microbiologic investigations for head and neck infections. Infect

Dis Clin North Am. 2007;21:283-304

Belleza WG, Kalman S. Otolaryngologic emergencies in the outpatient setting. Med Clin

North Am. 2006;90:329-53

Cannon ML, Antonio BL, McCloskey JJ, et al. Cavernous sinus thrombosis complicating

sinusitis. Pediatr Crit Care Med. 2004;5:86-8

Osborn MK, Steinberg JP. Subdural empyema and other suppurative complications of

paranasal sinusitis. Lancet Infect Dis. 2007;7:62-7

Codes

ICD-9 code

325 Phlebitis and thrombophlebitis of intracranial venous sinuses; embolism of

cavernous, lateral, or other intracranial or unspecified intracranial venous sinus;

endophlebitis of cavernous, lateral, or other intracranial or unspecified intracranial

venous sinus; phlebitis, septic or suppurative of cavernous, lateral, or other intracranial

or unspecified intracranial venous sinus; thrombophlebitis of cavernous, lateral, or other

intracranial or unspecified intracranial venous sinus; thrombosis of cavernous, lateral, or

other intracranial or unspecified intracranial venous sinus

437.6 Nonpyogenic thrombosis of intracranial venous sinus

FAQ

What is the basis of symptoms/findings in the condition? The structures that go

through the cavernous sinus, which are cranial nerves III, IV, VI (ophthalmoplegia), cranial

nerve V (facial paresthesia), internal carotid artery (unilateral weakness), and the

ophthalmic artery (unilateral blindness)

Should cavernous sinus thrombosis be suspected in all patients with unilateral

headaches ? Only if there is a history of a preceding infection in the face or sinuses and/or

there are suggestive eye findings on examination. However, in general, infection should be

considered in almost all patients who have a headache severe enough to require medical

attention

Are there predisposing factors for cavernous sinus thrombosis? As with other

cerebral venous thromboses, states of hypercoagulability, oral contraceptives, and diabetes

can predispose to cerebral venous thrombosis. Sinus infection is a more proximate risk

factor for development of cavernous sinus thrombosis

Can I prescribe oral contraceptive pills to someone with a history of cavernous

sinus thrombosis? No. Combined oral contraceptives represent an increased risk for

sinus thrombosis, especially in patients who have already had the condition


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