Cavernous sinus thrombosis
Encyclopedia
Cavernous sinus thrombosis (CST) is the formation of a blood clot within the cavernous sinus
, a cavity at the base of the brain which drains deoxygenated blood from the brain back to the heart. The cause is usually from a spreading infection in the nose, sinuses, ears, or teeth. Staphylococcus aureus
and Streptococcus
are often the associated bacteria. Cavernous sinus thrombosis symptoms include; decrease or loss of vision, chemosis
, exophthalmos
(bulging eyes), headaches, and paralysis of the cranial nerves which course through the cavernous sinus. This infection is life-threatening and requires immediate treatment, which usually includes antibiotic
s and sometimes surgical drainage.
The most common signs of CST are related to anatomical structures affected within the cavernous sinus, notably cranial nerves III-VI, as well as symptoms resulting from impaired venous drainage from the orbit and eye.
Classic presentations are abrupt onset of unilateral periorbital edema
, headache
, photophobia
, and bulging of the eye (proptosis).
Other common signs and symptoms include:
Ptosis
, chemosis
, cranial nelve palsies (III, IV, V, VI). Sixth nerve palsy is the most common. Sensory deficits of the ophthalmic and maxillary branch of the fifth nerve are common. Periorbital sensory loss and impaired corneal reflex may be noted. Papilledema
, retinal hemorrhages, and decreased visual acuity and blindness may occur from venous congestion within the retina. Fever
, tachycardia
and sepsis
may be present. Headache with nuchal rigidity may occur. Pupil
may be dilated and sluggishly reactive. Infection can spread to contralateral cavernous sinus
within 24–48 hours of initial presentation.
). The highly anastomotic venous system of the paranasal sinuses allows retrograde spread of infection to the cavernous sinus via the superior and inferior ophthalmic veins. It was previously thought that veins in the area were valveless and that this was the major cause of the retrograde spread, however, a recent study has found that the ophthalmic and facial veins are not valveless.
Staphylococcus aureus is the most common infectious microbe, found in 70% of the cases. Streptococcus is the second leading cause. Gram-negative rods and anaerobes
may also lead to cavernous sinus thrombosis. Rarely, Aspergillus fumigatus
and mucormycosis
cause CST.
, ESR
, blood cultures, and sinus cultures help establish and identify an infectious primary source. Lumbar puncture
is necessary to rule out meningitis.
, thickening of the superior ophthalmic vein, and irregular filling defects within the cavernous sinus; however, findings may be normal early in the disease course.
A MRI
using flow parameters and an MR venogram
are more sensitive than a CT scan, and are the imaging studies of choice to diagnose cavernous sinus thrombosis. Findings may include deformity of the internal carotid artery within the cavernous sinus, and an obvious signal hyperintensity within thrombosed vascular sinuses on all pulse sequences.
Cerebral angiography
can be performed, but it is invasive and not very sensitive. Orbital venography is difficult to perform, but it is excellent in diagnosing occlusion of the cavernous sinus.
, cavernous sinus thrombosis has a mortality rate of less than 20% in areas with access to antibiotics. Before antibiotics were available, the mortality was 80–100%. Morbidity rates also dropped from 70% to 22% due to earlier diagnosis and treatment.
Vancomycin
may be substituted for nafcillin if significant concern exists for infection by methicillin-resistant Staphylococcus aureus or resistant Streptococcus pneumoniae. Appropriate therapy should take into account the primary source of infection as well as possible associated complications such as brain abscess, meningitis, or subdural empyema
.
Anticoagulation with heparin
is controversial. Retrospective studies show conflicting data. This decision should be made with subspecialty consultation.
Steroid therapy is also controversial and is not recommended by many sources.
.
All patients with CST are usually treated with prolonged courses (3–4 weeks) of IV antibiotics. If there is evidence of complications such as intracranial suppuration, 6–8 weeks of total therapy may be warranted.
All patients should be monitored for signs of complicated infection, continued sepsis, or septic emboli while antibiotic therapy is being administered.
Complications of untreated CST include extension of thrombus to other dural venous sinuses, carotid thrombosis with concomitant strokes, subdural empyema, brain abscess, or meningitis. Septic embolization may also occur to the lungs, resulting in acute respiratory distress syndrome
, pulmonary abscess, empyema, and pneumothorax
.
Complications in treated patients include oculomotor weakness, blindness, pituitary insufficiency
, and hemiparesis
.
Cavernous sinus
The cavernous sinus , within the human head, is a large collection of thin-walled veins creating a cavity bordered by the temporal bone of the skull and the sphenoid bone, lateral to the sella turcica.-Contents:...
, a cavity at the base of the brain which drains deoxygenated blood from the brain back to the heart. The cause is usually from a spreading infection in the nose, sinuses, ears, or teeth. Staphylococcus aureus
Staphylococcus aureus
Staphylococcus aureus is a facultative anaerobic Gram-positive coccal bacterium. It is frequently found as part of the normal skin flora on the skin and nasal passages. It is estimated that 20% of the human population are long-term carriers of S. aureus. S. aureus is the most common species of...
and Streptococcus
Streptococcus
Streptococcus is a genus of spherical Gram-positive bacteria belonging to the phylum Firmicutes and the lactic acid bacteria group. Cellular division occurs along a single axis in these bacteria, and thus they grow in chains or pairs, hence the name — from Greek στρεπτος streptos, meaning...
are often the associated bacteria. Cavernous sinus thrombosis symptoms include; decrease or loss of vision, chemosis
Chemosis
Chemosis is the swelling of the conjunctiva. In general, chemosis is a nonspecific sign of eye irritation. The outer surface covering appears to have fluid in it. Often, the eye area swells so much that the eyes become difficult or impossible to close fully. It is usually caused by allergies or...
, exophthalmos
Exophthalmos
Exophthalmos is a bulging of the eye anteriorly out of the orbit. Exophthalmos can be either bilateral or unilateral . Measurement of the degree of exophthalmos is performed using an exophthalmometer...
(bulging eyes), headaches, and paralysis of the cranial nerves which course through the cavernous sinus. This infection is life-threatening and requires immediate treatment, which usually includes antibiotic
Antibiotic
An antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...
s and sometimes surgical drainage.
Clinical features
The clinical presentation of CST can be varied. Both acute, fulminant disease and indolent, subacute presentations have been reported in the literature.The most common signs of CST are related to anatomical structures affected within the cavernous sinus, notably cranial nerves III-VI, as well as symptoms resulting from impaired venous drainage from the orbit and eye.
Classic presentations are abrupt onset of unilateral periorbital edema
Edema
Edema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...
, headache
Headache
A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
, photophobia
Photophobia
Photophobia is a symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of actual physical photosensitivity of the eyes, though the term...
, and bulging of the eye (proptosis).
Other common signs and symptoms include:
Ptosis
Ptosis
Ptosis refers to droopiness of a body part. Specifically, it can refer to:* Ptosis * Ptosis * Enteroptosis * Gastroptosis...
, chemosis
Chemosis
Chemosis is the swelling of the conjunctiva. In general, chemosis is a nonspecific sign of eye irritation. The outer surface covering appears to have fluid in it. Often, the eye area swells so much that the eyes become difficult or impossible to close fully. It is usually caused by allergies or...
, cranial nelve palsies (III, IV, V, VI). Sixth nerve palsy is the most common. Sensory deficits of the ophthalmic and maxillary branch of the fifth nerve are common. Periorbital sensory loss and impaired corneal reflex may be noted. Papilledema
Papilledema
Papilledema is optic disc swelling that is caused by increased intracranial pressure. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare....
, retinal hemorrhages, and decreased visual acuity and blindness may occur from venous congestion within the retina. Fever
Fever
Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...
, tachycardia
Tachycardia
Tachycardia comes from the Greek words tachys and kardia . Tachycardia typically refers to a heart rate that exceeds the normal range for a resting heart rate...
and sepsis
Sepsis
Sepsis is a potentially deadly medical condition that is characterized by a whole-body inflammatory state and the presence of a known or suspected infection. The body may develop this inflammatory response by the immune system to microbes in the blood, urine, lungs, skin, or other tissues...
may be present. Headache with nuchal rigidity may occur. Pupil
Pupil
The pupil is a hole located in the center of the iris of the eye that allows light to enter the retina. It appears black because most of the light entering the pupil is absorbed by the tissues inside the eye. In humans the pupil is round, but other species, such as some cats, have slit pupils. In...
may be dilated and sluggishly reactive. Infection can spread to contralateral cavernous sinus
Cavernous sinus
The cavernous sinus , within the human head, is a large collection of thin-walled veins creating a cavity bordered by the temporal bone of the skull and the sphenoid bone, lateral to the sella turcica.-Contents:...
within 24–48 hours of initial presentation.
Etiology
CST most commonly results from contiguous spread of infection from the nasal furuncle (50%), sphenoidal or ethmoidal sinuses (30%) and dental infections (10%). Less common primary sites of infection include tonsils, soft palate, middle ear, or orbit (orbital cellulitisOrbital cellulitis
Orbital cellulitisis an infection of eye tissues posterior to the orbital septum. It most commonly refers to an acute spread of infection into the eye socket from either the adjacent sinuses or through the blood...
). The highly anastomotic venous system of the paranasal sinuses allows retrograde spread of infection to the cavernous sinus via the superior and inferior ophthalmic veins. It was previously thought that veins in the area were valveless and that this was the major cause of the retrograde spread, however, a recent study has found that the ophthalmic and facial veins are not valveless.
Staphylococcus aureus is the most common infectious microbe, found in 70% of the cases. Streptococcus is the second leading cause. Gram-negative rods and anaerobes
Anaerobic organism
An anaerobic organism or anaerobe is any organism that does not require oxygen for growth. It could possibly react negatively and may even die if oxygen is present...
may also lead to cavernous sinus thrombosis. Rarely, Aspergillus fumigatus
Aspergillus fumigatus
Aspergillus fumigatus is a fungus of the genus Aspergillus, and is one of the most common Aspergillus species to cause disease in individuals with an immunodeficiency....
and mucormycosis
Mucormycosis
Zygomycosis is the broadest term to refer to infections caused by bread mold fungi of the zygomycota phylum. However, because zygomycota has been identified as polyphyletic, and is not included in modern fungal classification systems, the diseases that Zygomycosis can refer to are better called by...
cause CST.
Diagnosis
The diagnosis of cavernous sinus thrombosis is made clinically, with imaging studies to confirm the clinical impression. Proptosis, ptosis, chemosis, and cranial nerve palsy beginning in one eye and progressing to the other eye establish the diagnosis.Differential diagnosis
- Orbital cellulitisOrbital cellulitisOrbital cellulitisis an infection of eye tissues posterior to the orbital septum. It most commonly refers to an acute spread of infection into the eye socket from either the adjacent sinuses or through the blood...
- Internal carotid arteryCarotid arteryCarotid artery can refer to:* Common carotid artery* External carotid artery* Internal carotid artery...
aneurysmAneurysmAn aneurysm or aneurism is a localized, blood-filled balloon-like bulge in the wall of a blood vessel. Aneurysms can commonly occur in arteries at the base of the brain and an aortic aneurysm occurs in the main artery carrying blood from the left ventricle of the heart... - Stroke
- MigraineMigraineMigraine is a chronic neurological disorder characterized by moderate to severe headaches, and nausea...
headacheHeadacheA headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the... - Allergic blepharitisBlepharitisBlepharitis is an ocular condition characterized by chronic inflammation of the eyelid, the severity and time course of which can vary. Onset can be acute, resolving without treatment within 2–4 weeks , but more generally is a long standing inflammation varying in severity...
- ThyroidThyroidThe thyroid gland or simply, the thyroid , in vertebrate anatomy, is one of the largest endocrine glands. The thyroid gland is found in the neck, below the thyroid cartilage...
exophthalmosExophthalmosExophthalmos is a bulging of the eye anteriorly out of the orbit. Exophthalmos can be either bilateral or unilateral . Measurement of the degree of exophthalmos is performed using an exophthalmometer... - Brain tumorBrain tumorA brain tumor is an intracranial solid neoplasm, a tumor within the brain or the central spinal canal.Brain tumors include all tumors inside the cranium or in the central spinal canal...
- MeningitisMeningitisMeningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...
- Mucormycosis
- TraumaPhysical traumaTrauma refers to "a body wound or shock produced by sudden physical injury, as from violence or accident." It can also be described as "a physical wound or injury, such as a fracture or blow." Major trauma can result in secondary complications such as circulatory shock, respiratory failure and death...
Workup
Cavernous sinus thrombosis is a clinical diagnosis with laboratory tests and imaging studies confirming the clinical impression.Laboratory tests
CBCComplete blood count
A complete blood count , also known as full blood count or full blood exam or blood panel, is a test panel requested by a doctor or other medical professional that gives information about the cells in a patient's blood...
, ESR
Erythrocyte sedimentation rate
The erythrocyte sedimentation rate , also called a sedimentation rate or Biernacki Reaction, is the rate at which red blood cells sediment in a period of 1 hour...
, blood cultures, and sinus cultures help establish and identify an infectious primary source. Lumbar puncture
Lumbar puncture
A lumbar puncture is a diagnostic and at times therapeutic procedure that is performed in order to collect a sample of cerebrospinal fluid for biochemical, microbiological, and cytological analysis, or very rarely as a treatment to relieve increased intracranial pressure.-Indications:The...
is necessary to rule out meningitis.
Imaging studies
Sinus films are helpful in the diagnosis of sphenoid sinusitis. Opacification, sclerosis, and air-fluid levels are typical findings. Contrast-enhanced CT scan may reveal underlying sinusitisSinusitis
Sinusitis is inflammation of the paranasal sinuses, which may be due to infection, allergy, or autoimmune issues. Most cases are due to a viral infection and resolve over the course of 10 days...
, thickening of the superior ophthalmic vein, and irregular filling defects within the cavernous sinus; however, findings may be normal early in the disease course.
A MRI
Magnetic resonance imaging
Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
using flow parameters and an MR venogram
Venography
Venography is a procedure in which an x-ray of the veins, a venogram, is taken after a special dye is injected into the bone marrow or veins. The dye has to be injected constantly via a catheter, making it an invasive procedure...
are more sensitive than a CT scan, and are the imaging studies of choice to diagnose cavernous sinus thrombosis. Findings may include deformity of the internal carotid artery within the cavernous sinus, and an obvious signal hyperintensity within thrombosed vascular sinuses on all pulse sequences.
Cerebral angiography
Cerebral angiography
Cerebral angiography is a form of angiography which provides images of blood vessels in and around the brain, thereby allowing detection of abnormalities such as arteriovenous malformations and aneurysms....
can be performed, but it is invasive and not very sensitive. Orbital venography is difficult to perform, but it is excellent in diagnosing occlusion of the cavernous sinus.
Treatment
According to the ICD-9M codeICD-9-CM Volume 3
ICD-9-CM Volume 3 is a system of procedural codes. It is a subset of ICD-9-CM - Procedures and interventions, not elsewhere classified :* Procedures and interventions, not elsewhere classified...
, cavernous sinus thrombosis has a mortality rate of less than 20% in areas with access to antibiotics. Before antibiotics were available, the mortality was 80–100%. Morbidity rates also dropped from 70% to 22% due to earlier diagnosis and treatment.
Non-pharmacologic therapy
Recognizing the primary source of infection (i.e., facial cellulitis, middle ear, and sinus infections) and treating the primary source expeditiously is the best way to prevent cavernous sinus thrombosis.Acute general treatment
Broad-spectrum intravenous antibiotics are used until a definite pathogen is found.- Nafcillin 1.5 g IV q4h
- CefotaximeCefotaximeCefotaxime is a third-generation cephalosporin antibiotic. Like other third-generation cephalosporins, it has broad spectrum activity against Gram positive and Gram negative bacteria...
1.5 to 2 g IV q4h - MetronidazoleMetronidazoleMetronidazole is a nitroimidazole antibiotic medication used particularly for anaerobic bacteria and protozoa. Metronidazole is an antibiotic, amebicide, and antiprotozoal....
15 mg/kg load followed by 7.5 mg/kg IV q6h
Vancomycin
Vancomycin
Vancomycin INN is a glycopeptide antibiotic used in the prophylaxis and treatment of infections caused by Gram-positive bacteria. It has traditionally been reserved as a drug of "last resort", used only after treatment with other antibiotics had failed, although the emergence of...
may be substituted for nafcillin if significant concern exists for infection by methicillin-resistant Staphylococcus aureus or resistant Streptococcus pneumoniae. Appropriate therapy should take into account the primary source of infection as well as possible associated complications such as brain abscess, meningitis, or subdural empyema
Subdural empyema
It is a form of empyema in the subdural space.Bacterial or occasionally fungal infection of the skull bones or air sinuses can spread to the subdural space, producing a subdural empyema. The underlying arachnoid and subarachnoid spaces are usually unaffected, but a large subdural empyema may...
.
Anticoagulation with heparin
Heparin
Heparin , also known as unfractionated heparin, a highly sulfated glycosaminoglycan, is widely used as an injectable anticoagulant, and has the highest negative charge density of any known biological molecule...
is controversial. Retrospective studies show conflicting data. This decision should be made with subspecialty consultation.
Steroid therapy is also controversial and is not recommended by many sources.
Long-term treatment
Surgical drainage with sphenoidotomy is indicated if the primary site of infection is thought to be the sphenoidal sinusesSphenoidal sinuses
The two sphenoidal sinuses contained within the body of the sphenoid vary in size and shape; owing to the lateral displacement of the intervening septum they are rarely symmetrical....
.
All patients with CST are usually treated with prolonged courses (3–4 weeks) of IV antibiotics. If there is evidence of complications such as intracranial suppuration, 6–8 weeks of total therapy may be warranted.
All patients should be monitored for signs of complicated infection, continued sepsis, or septic emboli while antibiotic therapy is being administered.
Key points
Cavernous sinus thrombosis can be a life-threatening, rapidly progressive infectious disease with high morbidity and mortality rates despite antibiotic use.Complications of untreated CST include extension of thrombus to other dural venous sinuses, carotid thrombosis with concomitant strokes, subdural empyema, brain abscess, or meningitis. Septic embolization may also occur to the lungs, resulting in acute respiratory distress syndrome
Acute respiratory distress syndrome
Acute respiratory distress syndrome , also known as respiratory distress syndrome or adult respiratory distress syndrome is a serious reaction to various forms of injuries to the lung....
, pulmonary abscess, empyema, and pneumothorax
Pneumothorax
Pneumothorax is a collection of air or gas in the pleural cavity of the chest between the lung and the chest wall. It may occur spontaneously in people without chronic lung conditions as well as in those with lung disease , and many pneumothoraces occur after physical trauma to the chest, blast...
.
Complications in treated patients include oculomotor weakness, blindness, pituitary insufficiency
Hypopituitarism
Hypopituitarism is the decreased secretion of one or more of the eight hormones normally produced by the pituitary gland at the base of the brain...
, and hemiparesis
Hemiparesis
Hemiparesis is weakness on one side of the body. It is less severe than hemiplegia - the total paralysis of the arm, leg, and trunk on one side of the body. Thus, the patient can move the impaired side of his body, but with reduced muscular strength....
.