CareWorks Health Services
Moulton Parkway STE 103C
Laguna Hills, CA 92653
venous sinus thrombosis
(CVST) is the presence of thrombosis
(a blood clot) in the dural
venous sinuses, which drain blood from the brain.
Symptoms may include headache,
abnormal vision, any of the symptoms of stroke
such as weakness of the face and limbs on one side of the
body, and seizures.
The diagnosis is usually by computed
tomography (CT/CAT scan) or magnetic
resonance imaging (MRI) employing radiocontrast
to demonstrate obstruction of the venous sinuses by thrombus.
is with anticoagulants
(medication that suppresses blood clotting), and rarely
(enzymatic destruction of the blood clot). Given that there
is usually an underlying cause for the disease, tests may
be performed to look for these. The disease may be complicated
intracranial pressure, which may warrant surgical intervention
such as the placement of a shunt.
There are several other terms for the condition, such as
cerebral venous and sinus thrombosis, (superior)
sagittal sinus thrombosis, dural sinus thrombosis
and intracranial venous thrombosis as well as the
older term cerebral thrombophlebitis.
in ten people with sinus thrombosis have a headache; this
tends to worsen over the period of several days, but may
also develop suddenly (thunderclap
The headache may be the only symptom of cerebral venous
Many patients have symptoms of stroke: inability to move
one or more limbs, weakness on one side of the face or difficulty
speaking. This does not necessarily affect one side
of the body as in the more common "arterial" stroke.
of all patients have seizures,
although it is more common still in women who develop sinus
thrombosis peripartum (in the period before and after giving
These are mostly seizures affecting
only one part of the body and unilateral
(occurring on one side), but occasionally the seizures are
and rarely they lead to status
epilepticus (persistent or recurrent seizure activity
for a long period of time).
the elderly, many of the aforementioned symptoms may not
occur. Common symptoms in the elderly with this condition
are otherwise unexplained changes in mental
status and a depressed level
around the brain may rise, causing papilledema
(swelling of the optic
disc) which may be experienced as visual obscurations.
In severely raised intracranial pressure, the level of consciousness
is decreased, the blood
pressure rises, the heart
rate falls and the patient assumes an abnormal posture.
venous sinus thrombosis is more common in particular situations.
85% of patients have at least one of these risk factors:
a tendency to develop blood clots due to abnormalities
in coagulation, e.g. factor
V Leiden, deficiency of protein
S or antithrombin,
or related problems
syndrome, a kidney problem causing protein loss
in the urine
inflammatory diseases, such as inflammatory
bowel disease, lupus
(the period after giving birth)
blood disorders, especially polycythemia
vera and paroxysmal
of estrogen-containing forms of hormonal
and infections of the ear, nose and throat area such
injury to the venous sinuses
procedures in the head and neck area
primarily in infants and children
diagnosis may be suspected on the basis of the symptoms,
for example the combination of headache, signs of raised
pressure and focal neurological abnormalities, or when
alternative causes of headache and neurological abnormalities,
such as a subarachnoid
hemorrhage, have been excluded.
venogram showing a filling defect in the sagittal
sinus (black arrow)
are various neuroimaging
investigations that may detect cerebral sinus thrombosis.
edema and venous infarction may be apparent on any modality,
but for the detection of the thrombus itself, the most commonly
used tests are computed
tomography (CT) and magnetic
resonance imaging (MRI), both using various types of
radiocontrast to perform a venogram
and visualise the veins around the brain.
tomography, with radiocontrast
in the venous phase (CT venography or CTV), has a
detection rate that in some regards exceeds that of MRI.
The test involves injection into a vein (usually in the
arm) of a radioopaque substance, and time is allowed for
the bloodstream to carry it to the cerebral veins - at which
point the scan is performed. It has a sensitivity
of 75-100% (it detects 75-100% of all clots present), and
of 81-100% (it would be incorrectly positive in 0-19%).
In the first two weeks, the "empty delta sign" may be observed
(in later stages, this sign may disappear).
resonance venography employs the same principles, but
uses MRI as a scanning modality. MRI has the advantage of
being better at detecting damage to the brain itself as
a result of the increased pressure on the obstructed veins,
but it is not readily available in many hospitals and the
interpretation may be difficult.
angiography may demonstrate smaller clots than CT or
MRI, and obstructed veins may give the "corkscrew appearance".
This, however, requires puncture of the femoral
artery with a sheath and advancing a thin tube through
the blood vessels to the brain where radiocontrast is injected
before X-ray images are obtained. It is therefore only performed
if all other tests give unclear results or when other treatments
may be administered during the same procedure.
2004 study suggested that the D-dimer
blood test, already in use for the diagnosis of other forms
of thrombosis, was abnormal (above 500 microg/l) in 34 out
of 35 patients with cerebral sinus thrombosis, giving it
of 97.1%, a negative
predictive value of 99.6%, a specificity
of 91.2%, and a positive predictive value of 55.7%. Furthermore,
the level of the D-dimer correlated with the extent of the
A subsequent study, however, showed that 10% of patients
with confirmed thrombosis had a normal D-dimer, and in those
who had presented with only a headache 26% had a normal
D-dimer. The study concludes that D-dimer is not useful
in the situations where it would make the most difference,
namely in lower probability cases.
most patients, the direct cause for the cerebral sinus thrombosis
is not readily apparent. Identifying a source of infection
is crucial; it is common practice to screen for various
forms of thrombophilia
(a propensity to form blood clots).
veins of the brain,
both the superficial veins and the deep venous system, empty
into the dural venous sinuses, which carry blood back to
vein and thence to the heart.
In cerebral venous sinus thrombosis, blood clots usually
form both in the veins of the brain and the venous sinuses.
The thrombosis of the veins themselves causes venous infarctiondamage
to brain tissue due to a congested
and therefore insufficient blood supply. This results in
edema (both vasogenic and cytotoxic edema),
and leads to small petechial
haemorrhages that may merge into large haematomas. Thrombosis
of the sinuses is the main mechanism behind the increase
in intracranial pressure due to decreased resorption of
cerebrospinal fluid (CSF). The condition does not lead to
however, because there is no difference in pressure between
various parts of the brain.
blood clot forms due to an imbalance between coagulation
(the formation of the insoluble blood protein fibrin)
The three major mechanisms for such an imbalance are enumerated
triad: alterations in normal blood flow, injury to the
blood vessel wall, and alterations in the constitution of
blood (hypercoagulability). Most cases of cerebral venous
sinus thrombosis are due to hypercoagulability.
is possible for the clot to break off and migrate (embolise)
to the lungs, causing
An analysis of earlier case reports concludes that this
occurs in about 10% of cases, but has a very poor prognosis.
studies have investigated the use of anticoagulation
to suppress blood clot formation in cerebral venous sinus
thrombosis. Before these trials had been conducted, there
had been a concern that small areas of hemorrhage in the
brain would bleed further as a result of treatment; the
studies showed that this concern was unfounded.
practice guidelines now recommend heparin
molecular weight heparin in the initial treatment, followed
provided there are no other bleeding risks that would make
these treatments unsuitable.
Some experts discourage the use of anticoagulation if there
is extensive hemorrhage; in that case, they recommend repeating
the imaging after 710 days. If the hemorrhage has
decreased in size, anticoagulants are commenced, while no
anticoagulants are given if there is no reduction.
duration of warfarin treatment depends on the circumstances
and underlying causes of the condition. If the thrombosis
developed under temporary circumstances (e.g. pregnancy),
three months are regarded as sufficient. If the condition
was unprovoked but there are no clear causes or a "mild"
form of thrombophilia, 6 to 12 months is advised. If there
is a severe underlying thrombosis disorder, warfarin treatment
may need to continue indefinitely.
(removal of the blood clot with "clot buster" medication)
has been described, either systemically by injection into
a vein or directly into the clot during angiography. The
Federation of Neurological Societies guideline recommends
that thrombolysis is only used in patients who deteriorate
despite adequate treatment, and other causes of deterioration
have been eliminated. It is unclear which drug and which
mode of administration is the most effective. Bleeding into
the brain and in other sites of the body is a major concern
in the use of thrombolysis.
American guidelines make no recommendation with regards
to thrombolysis, stating that more research is needed.
intracranial pressure, if severe or threatening vision,
may require therapeutic lumbar
puncture (removal of excessive cerebrospinal
fluid), medication (acetazolamide),
treatment (optic nerve sheath fenestration or shunting).
In certain situations, anticonvulsants
may be used to prevent seizures; these are focal neurological
problems (e.g. inability to move a limb) and/or focal changes
of the brain tissue on CT or MRI scan.
2004 the first adequately large scale study on the natural
history and long-term prognosis of this condition was reported;
this showed that at 16 months follow-up 57.1% of patients
had full recovery, 29.5%/2.9%/2.2% had respectively minor/moderate/severe
symptoms or impairments, and 8.3% had died. Severe impairment
or death were more likely in those aged over 37 years, male,
affected by coma, mental status disorder, intracerebral
hemorrhage, thrombosis of the deep cerebral venous system,
central nervous system infection and cancer.
A subsequent systematic
review of nineteen studies in 2006 showed that mortality
is about 5.6% during hospitalisation and 9.4% in total,
while of the survivors 88% make a total or near-total recovery.
After several months, two thirds of the cases has resolution
("recanalisation") of the clot. The rate of recurrence was
children with CVST, the mortality averages 50%. Poor outcome
is more likely if a child with CVST develops seizures or
has evidence of venous infarction on imaging.
venous sinus thrombosis is rare, with an estimated 3-4 cases
per million annual incidence in adults. While it may occur
in all age groups, it is most common in the third decade.
75% are female.
Given that older studies show no difference in incidence
between men and women, it has been suggested that the use
contraceptives in women is behind the disparity between
A 1995 report from Saudi
Arabia found a doubled incidence at 7 cases per 100,000;
this was attributed to the fact that Behçet's
disease, which increases risk of CVST, is more common
in the Middle East.
1973 report found that CVST could be found on autopsy
(examination of the body after death) in nine percent of
all people. Many of these were elderly and had neurological
symptoms in the period leading up to their death, and many
suffered from concomitant heart
children, a Canadian study reported in 2001 that CVST occurs
in 6.7 per million annually. 43% occur in the newborn (less
than one month old), and a further 10% in the first year
of life. Of the newborn, 84% were already ill, mostly from
complications after childbirth and dehydration.
first description of thrombosis of the cerebral veins and
sinuses is attributed to the French physician Ribes, who
in 1825 observed thrombosis of the saggital sinus and cerebral
veins in a man who had suffered from seizures and delirium.
Until the second half of the 20th century it remained a
diagnosis generally made after death.
In the 1940s, reports by Dr Charles
Symonds and others allowed for the clinical diagnosis
of cerebral venous thrombosis, using characteristic signs
and symptoms and results of lumbar
on the diagnosis of cerebral venous sinus thrombosis in
life were made with the introduction of venography
which also aided in the distinction from idiopathic
which has similar presenting signs and symptoms in many
British gynecologist Stansfield is credited with the introduction,
in 1942, of the just recently introduced anticoagulant heparin
in the treatment of CVST in 1942.
Clinical trials in the 1990s finally resolved the concern
about using anticoagulants in most cases of CVST.
Secretary of State Hillary
Rodham Clinton was hospitalized on December 30, 2012,
for anticoagulation treatment of venous thrombosis of the
sinus, which is located at the base of the brain. Clinton's
thrombotic episode was discovered on an MRI
scan done for follow-up of a cerebral
concussion she had suffered 2.5 weeks before after she
fell while suffering from gastroenteritis.
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