Isolated Lateral Sinus Thrombosis

Isolated Lateral Sinus Thrombosis
A Series of 62 Patients
Mariem Damak, MD; Isabelle Crassard, MD; Valérie Wolff, MD; Marie-Germaine Bousser, MD
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Background and Purpose—Isolated lateral sinus thrombosis was long considered a complication of middle ear disease.
Little attention has been recently paid to this variety of thrombosis. We therefore reviewed all cases of isolated lateral
sinus thrombosis prospectively collected in our center (1997 to 2006).
Methods—Among 195 patients with cerebral venous thrombosis (CVT), we identified 157 patients with lateral sinus
thrombosis, including 62 patients with isolated lateral sinus thrombosis. Clinical, etiologic, and prognostic features were
compared with those of other 133 CVT cases.
Result—Sixty-two patients (32%) had isolated lateral sinus thrombosis. Headaches were present in 95% of patients. The
main clinical presentation was isolated headache in 28 patients (45%), whereas 15 (24%) had isolated intracranial
hypertension. Nineteen patients (31%) had at least one focal sign (deficit and/or focal seizure). Dysphasia was the most
common one (8 patients). Compared with the other 133 CVT cases, presentation with isolated headaches was the most
frequent one (P⬍0.001). Parenchymal lesions were found in 19 cases and were less frequent than in other CVT cases
(P⫽0.007). Numerous causes or predisposing factors were identified without a difference in repartition with other
patients with CVT, particularly for local or infectious causes. Treatment consisted of anticoagulation in all patients.
Clinical outcome was good with complete recovery in 57 patients (92%). One patient died after the occurrence of massive
pulmonary emboli despite adequate anticoagulation. Three patients had sequelae (no difference with the other CVT).
Conclusion—Isolated lateral sinus thrombosis is a frequent variety of CVT, accounting for one third of all CVT. It presents
in more than two thirds of cases with headache as the only symptom either isolated or less frequently associated with
papilledema. This stresses the need for CVT workup in a patient with a recent unexplained headache. (Stroke.
2009;40:476-481.)
Key Words: headache 䡲 lateral sinus 䡲 sinus thrombosis
C
since the widespread use of neuroimaging, the pattern of
presentation of isolated LST is likely to have changed.
We describe here the main clinical and MRI features, the
causes and outcome of all patients with isolated LST prospectively collected in our center in the past 10 years, and
compare them with those of the other varieties of CVT
examined during the same period.
erebral venous thrombosis (CVT) is an infrequent condition characterized by a wide spectrum of clinical
presentations and modes of onset, which depend on the site,
extent, and rate of progression of thrombosis.1,2
The 2 most frequent sites of thrombosis are the superior
sagittal sinus (SSS), affected in 62% to 80% of cases, and the
lateral sinus (LS) involved in 38% to 86% of cases.1,3–5 A
remarkable feature of CVT is that, in approximately 75%
of cases, thrombosis affects several sinuses, the most
frequent combination being SSS ⫹ LS.1 The isolated
involvement of one sinus is thus rare, less then 30% for
SSS and 10% for LS.1,3,4
Isolated LS thrombosis (LST) has mostly been reported in
otologic series6 – 8 as a consequence of acute or chronic ear
and mastoid infections, hence the term “otitic hydrocephalus”
coined by Symonds when LST manifested as raised intracranial pressure.9
Because of, first, the dramatic reduction of intracranial
complications of infectious ear diseases since the antibiotic
era, and second, the possibility of early diagnosis of CVT
Patients and Methods
The present study is based on a prospective cohort of 195 consecutive patients with CVT admitted to our department between September 1997 and April 2006.
Diagnosis of CVT was based on MRI combined with MR
venography and/or helical cerebral CT venography. The diagnosis of
LST was based on the association of negative (nonvisualization of
the entire LS or of its sigmoid part at CT angiography or MR
venography) and positive signs (definite spontaneous LS hyperdensity on nonenhanced CT or hypersignal on MRI T1 or T2 WI or
hyposignal on MRI T2*SW)2,10,11 (Figure 1). The presence of
parenchymal lesions was assessed on both nonenhanced CT scan and
MRI (T1, T2, fluid-attenuated inversion recovery, diffusion, and
T2*SW).
Received November 16, 2007; final revision received February 12, 2008; accepted February 27, 2008.
From the Neurology Department, Lariboisière Hospital, Paris, France.
Correspondence to Isabelle Crassard, MD, Neurology Department, Lariboisière Hospital, 2 rue Ambroise Paré, 75010 Paris, France. E-mail
[email protected]
© 2009 American Heart Association, Inc.
Stroke is available at http://stroke.ahajournals.org
DOI: 10.1161/STROKEAHA.107.509711
476
Damak et al
Isolated Lateral Sinus Thrombosis
477
Table 1. Characteristics, Mode of Onset of Thrombosis,
Presenting Symptoms, and Signs in Patients With Isolated LST
versus Other Sites of CVT*
Isolated
LST
(n⫽62)
Other CVT
(n⫽133)
n
(%)
n
(%)
50
(81)
105
P
Patients
Female sex
Mean age (SD)
38 years
(14)
(80)
38 years
(12.5)
Onset
Acute
6
(10)
36
(27)
50
(80)
90
(68)
6
(10)
7
(5)
Headache
59
(95)
129
(96)
0.52
Papilledema
14
(22)
61
(46)
0.0033
Seizure
⬍0.0001
Subacute
Chronic
Signs and symptoms
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Figure 1. LST. A, Nonenhanced CT: spontaneous hyperdensity
of the lateral sinus. B, T1-weighted imaging: LS hypersignal. C,
T2* imaging: LS hyposignal. D, MR venography: absence of flow
in left LS.
LST was defined as isolated when, at admission in our department,
there was no associated SSS, deep venous system, or straight sinus
thrombosis. Patients with cortical vein thrombosis were also excluded except when the vein afferent to the thrombosed LS was the
only cortical vein involved.
After January 2001, mastoid air sinus abnormalities consisting of
increased T2 WI MRI signal in the mastoid air space12 were
systematically looked for and recorded.
Symptoms and signs, presence or absence of papilledema, modes
of onset, neuroimaging findings, causes and risk factors, including a
thrombophilic workup, and outcome at discharge and at 1 year were
systematically recorded. The onset was defined as acute (ⱕ2 days),
subacute (2 days to 1 month), or chronic (over 1 month).3 All
patients were treated acutely by a therapeutic dose of intravenous
heparin or low-molecular-weight heparin. Disability at discharge and
at 1 year was classified according to the modified Rankin Scale
(mRS) as complete recovery (mRS 0 to 1), partial recovery (mRS 2),
dependent (mRS 3 to 5), or death (mRS 6).
Statistical Analysis
Patients with isolated LST were compared with the rest of CVT
cohort using the ␹2 test for dichotomous data and Student t test for
continuous data. All significance levels reported were 2-sided and a
probability value of 0.05 was considered to indicate statistical
significance.
Results
Baseline Clinical Characteristics
Among the 195 patients with CVT, 108 (55%) had SSS
thrombosis and 157 (80%) had LST. The present study is
based on 62 (32%) who had isolated LST and who were
compared with the other 133 patients.
Thrombosis affected the left LS in 36 patients (58%), the
right LS in 25 (40%), and both in one patient (2%).
The female:male sex ratio (4:1) and the mean age (38
years) were the same in the 2 groups. The onset was more
10
(16)
62
(47)
Dysphasia
8
(13)
17
(13)
0.98
Motor deficit
2
(3)
45
(34)
⬍0.0001
Sensory deficit
1
(2)
7
(5)
0.41
Homonymous
hemianopia
2
(3)
2
(1.5)
0.80
Altered mental status
3
(5)
37
(28)
0.0002
(13.5)
0.0055
Diffuse encephalopathy
0
(0)
18
Cerebellar ataxia
1
(2)
0
(0)
Hearing loss
2
(3)
3
(2)
Vertigo
3
(5)
0
(0)
VIth cranial nerve palsy
3
(5)
18
(14)
0.11
VIIth cranial nerve palsy
1
(2)
3
(2)
0.99
0.99
*Patients may have more than one symptom.
frequently subacute (80%) or chronic (10%) and less frequently acute (10%) than in other CVT.
Presenting symptoms and signs are listed in Table 1.
Headache was in both groups by far the most frequent
presenting symptom (95% and 96%, respectively), but papilledema was less frequent (22%) in isolated LST than in
other CVT (47%). Motor deficits, seizures, altered mental
status, and encephalopathy were far less frequent in isolated
LST than in other CVT. By contrast, the frequency of
dysphasia was the same (13%) in both groups. Other signs
such as cerebellar ataxia, hearing loss, vertigo, and VIth or
VIIth cranial nerve palsy were rare in both groups.
The main patterns of presentation are listed in Table 2.
Nearly half the patients (45%) with isolated LST presented
with headache only, far more than in patients with other CVT
(9%). A focal syndrome with focal deficits and/or seizures
was less frequent in patients with isolated LST (31%) than in
patients with other CVT (63%). Roughly one fourth of the
patients in both groups had isolated intracranial hypertension.
Neuroimaging
Initial noncontrast CT scan was performed on an emergency
basis in 57 patients (92%). It was normal in 23 cases (40%).
478
Stroke
February 2009
Table 2. Patterns of Presentation in Patients With Isolated
LST versus Other Sites of CVT
Isolated
LST
(n⫽62)
Other CVT
(n⫽133)
Local causes (infectious or not)
Gyneco-obstetric causes
n
(%)
n
(%)
P
Isolated headache
28
(45)
12
(9)
⬍0.0001
Isolated intracranial hypertension
15
(24)
36
(28)
Focal syndrome⫾encephalopathy
Diffuse encephalopathy
19
0
(31)
(0)
Table 3. Causes and Risk Factors in Patients With Isolated
LST versus Other Sites of CVT*
83
19
Isolated LST
(n⫽62) (%)
Other CVT
(n⫽133) (%)
6 (10)
8 (6)
27 (54)
52 (49)
3 (6)
18 (17)
40 (64)
70 (52)
Current oral contraceptive use
Pregnancy or puerperium
(63)
General diseases, coagulation disorders
(14)
Coagulation disorders, thrombophilia
25
35
Systemic disorders
17
26
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It showed definite spontaneous hyperdensity of the lateral
sinus in 26 patients (45%; Figure 1). Other abnormalities
were identified in 15 patients: parenchymal hypodensity in 6,
parenchymal hyperdensity or mixture of hypo- and hyperdensity in 8, associated with sulcal hemorrhage in 2, isolated
sulcal hemorrhage in one.
MRI with MR venography was performed in 58 patients
(94%) showing by definition the positive and negative signs
of LST. Other abnormalities were parenchymal lesions in 18
(31%) mostly in the temporal lobe (80%; Figure 2). Ten were
nonhemorrhagic and 8 were hemorrhagic, including 2 associated with sulcal hemorrhage. One patient had isolated sulcal
hemorrhage. Overall, patients with isolated LST had less
neuroimaging lesions (31%) than patients with other sites of
CVT (51%; P⫽0.007). Mastoid air sinus abnormalities on
Cancer and malignant hemopathies
More than one cause or risk factor
3
7
23 (37)
34 (26)
8 (13)
17 (13)
None identified
*Patients may have more than one risk factor.
MRI T2 WI, prospectively looked for in 39 patients, were
present in 11 patients (28%) invariably ipsilateral to LST
(Figure 2C).
Clinicoradiological Correlations
Among the 28 patients with isolated headache as the initial
presentation, 17 had a left LST and 11 a right LST. All but
2 (93%) had otherwise normal neuroimaging examinations. One had an isolated sulcal hemorrhage visible on
both CT scan and MRI fluid-attenuated inversion recovery
imaging. The other had a small parenchymal lesion on
MRI fluid-attenuated inversion recovery imaging in the
left temporal lobe.
In the 15 patients with isolated intracranial hypertension, only one had bilateral LST. In the 14 others, LST was
unilateral, on the left side in 6, on the right in 8, and it was
associated with contralateral hypoplasia and/or extension
to the torcular. Three patients had small parenchymal
involvement consisting of right temporal lesions in 2 and
left temporal in one.
Among the 19 patients with focal deficits or seizures, 13
had a left LST and 6 a right LST. A parenchymal lesion was
detected in 14 of them (74%), mostly in the temporal lobe.
Causes and Predisposing Factors
Causes and predisposing factors are summarized in Table 3.
No significant difference between isolated LST and other
CVT was found regarding causes and risk factors, even when
considering local causes (6% versus 10%). Only one patient
with isolated LST had otitis media, 2 had tonsillar abscess,
one had a homolateral cholesteatoma, one had a homolateral
bone hemangioma, and one had LST after surgery for cervical
syringomyelia.
Figure 2. Different intracranial lesions secondary to lateral sinus
thrombosis. A, Fluid-attenuated inversion recovery imaging: right
temporal edema. B, Nonenhanced CT: mixture of hypo- and
hyperdensity in the left temporal lobe. C, Coronal T2-weighted
imaging: increased T2-weighted MRI signal in the mastoid air
spaces homolateral to the lateral sinus hypersignal (thin arrow).
D, Fluid-attenuated inversion recovery imaging: left temporal
hematoma with mass effect.
Treatment and Outcome
Besides anticoagulation, additional treatments were prescribed according to the clinical presentation and causes:
antiepileptic drugs in patients with seizures and acetazolamide and/or lumbar puncture in patients with isolated
intracranial hypertension. The 3 patients with local infection
received antibiotics; one patient had surgical treatment for
Damak et al
Table 4. Clinical Outcome at Discharge and at 1-Year
Follow-Up
Isolated LST (n⫽62)
Other CVT (n⫽133)
At Discharge
At 1 Year
At Discharge
At 1 Year
53 (85%)
57 (93%)
89 (67%)
119 (91%)
Partial recovery
(mRS 2)
5 (8%)
2 (3%)
29 (22%)
8 (6%)
Dependent
(mRS 3–5)
3 (5%)
1* (2%)
14 (11%)
3 (2%)
Death (mRS 6)
1 (2%)
1 (2%)
1 (1%)
1 (1%)
Complete recovery
(mRS 0 –1)
*mRS⫽3.
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tonsillar abscess. One patient underwent a temporal hematoma evacuation on an emergency basis because of a severe
mass effect.
Disability at discharge and at 1 year is indicated in Table 4.
The overall prognosis was good with 85% of patients having
an mRS of 0 or 1 at discharge. This was significantly better
than for the other varieties of CVT. At 1 year, there was no
difference between the 2 groups with, respectively, 92% and
91% of patients with an mRS of 0 to 1.
Among the 62 patients with isolated LST, 5 had at
discharge an mRS of 2 and 3 had an mRS of 3 to 5. At 1 year,
2 patients had an mRS of 2 because of diminished vision and
one an mRS of 3 because of motor deficit and dysphasia. One
patient died during the acute phase. She worsened rapidly
after admission and developed temporal hematoma. Local
thrombectomy was performed but the patient did not regain
consciousness. A decompressive craniectomy was then performed. The patient started to improve but 6 days later, she
died of massive pulmonary emboli despite adequate
anticoagulation.
Discussion
In this prospective series of 195 patients with CVT seen in the
last 10 years in our institution, LS was the most frequently
involved sinus (80% versus 55% for SSS) and it was also the
most frequently involved in isolation, ie, without other
sinuses involved (32% versus 9% for SSS). This high
frequency of LST compared with SSS thrombosis4,13,14 raises
the question of LST diagnosis. We think overdiagnosis of
LST in our study is unlikely because LST diagnosis was
based on the association of the visualization of the thrombus
itself (hyperdensity on CT scan, hypersignal on MRI T1 and
T2, hyposignal on MRI T2*) and the nonvisualization of the
entire LS or its sigmoid part on angiography (MR venography, CT angiography, conventional angiography).2 The 2
main diagnostic pitfalls of LST, ie, LS hypoplasia and
arachnoid granulation, have thus been carefully ruled out. LS
hypoplasia can indeed be mistaken for LST if the diagnosis is
based purely on the absence of flow on angiography but not
if the positive image of the thrombus is detected in the
nonvisualized vessel.11 Similarly, arachnoid granulations,
particularly when they are large, can be misinterpreted as a
focal thrombosis if the diagnosis is based purely on the
presence of a “positive” image within the sinus but not if
Isolated Lateral Sinus Thrombosis
479
there is an absence of flow in the entire LS or its sigmoid
part.14
Our series of isolated LST differs from previous large
series, which were collected in ear, nose, and throat departments6 – 8,15–18 where LST most frequently occurred as a
complication of otitis media or other local infection.7,8,15–21
The typical pattern was that of patients with an old or recent
history of discharging ears who had neglected otitis media
and presented with retroauricular swelling, otorrhea, fever,
headache, nausea, and vomiting. In old series, patients were
treated by internal jugular vein ligation19,20 and, in more
recent ones, by antibiotics and eventually surgery.7,21 In our
series, only 3 patients had a local ear, nose, and throat
infectious cause (one otitis media, 2 tonsillar abscesses), not
more than other varieties of CVT. This low rate of local
infectious causes is consistent with recent series coming from
countries where antibiotics are widely used.3,13 A recruitment
bias is unlikely because our institution has the only ear, nose,
and throat emergency department for adults open 24 hours for
the whole Paris area. Although today rare, local causes—
infectious or not—should be systematically looked for by an
ear, nose, and throat examination in patients with LST.
When comparing isolated LST with other CVT, there was
no difference regarding age, sex, and the main causes; the
main difference pertains to the clinical presentation with
more isolated headache and less encephalic signs. Headache
was the only sign in 45% of patients with isolated LST
compared with 10% in other CVT. This unusually high rate of
isolated headache might be partly due to the presence in our
institution of an emergency headache center recruiting 8000
patients per year and allowing prompt investigations for
patients with unexplained recent headache.22
Among patients with isolated headache, 94% had a normal
brain MRI. The exact mechanism of headache in such
patients who have no intracranial hypertension, subarachnoid
hemorrhage, meningitis, or intracranial lesion remains unknown. A local inflammatory reaction is a possibility with
dilatation of vessels in the sinus walls as suggested by the
frequent contrast enhancement surrounding the clot, known
for SSS thrombosis as the “empty delta sign,” but also present
in LST.22 The pain might also be due to the irritation or
stretching of nerve fibers in the walls of the occluded sinus,
which often becomes round with a bulging aspect.23
Isolated intracranial hypertension resumed the clinical
presentation in approximately one fourth of patients with
isolated LST as well as with other CVT. Such presentation is
one of the most classical ones in CVT, particularly when
several sinuses are occluded without involvement of cerebral
veins.13,24,25 In our series, it was mostly observed when the
thrombosed LS was the dominant one, with hypoplasia of the
other, or when thrombosis involved the torcular or, in one
case, both LS.
Encephalic signs such as seizures, focal deficits, and
disorders of consciousness were less frequent (one third) in
isolated LST than in other CVT (two thirds). These differences in presentation are related to a significant difference in
the frequency of brain lesions: 31% in isolated LST versus
51% in other CVT. The main focal sign was dysphasia due to
a left temporal ischemic or hemorrhagic lesion secondary to
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February 2009
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thrombosis of the vein of Labbé, which drains in LS. Its
frequency (13%) was the same as in other CVT. Motor deficit
(3%) was 10 times less frequent than in other CVT and
seizures (16%) 3 times less. No patient presented with diffuse
subacute encephalopathy. Previous reports suggest that LST
can manifest as isolated or multiple cranial nerve palsy.26,27
This was rare in our series; one patient had facial palsy and 2
had hearing loss, adding to a previous case report.28 Hearing
loss may be due to the extension of thrombosis to cochlear
veins, which empty into the lateral sinus directly or through
the inferior petrosal vein.
Regarding imaging, attention has recently been drawn to
the possibility in patients with LST of ipsilateral mastoid
abnormalities with increased T2-weighted signal in the
mastoid air spaces, often with a trabecular pattern.12 Such
mastoid abnormalities were present in 39% of patients with
LST in Fink’s original description and in 28% in our
series, but not in the 3 patients who had an infectious local
cause. When present, they should not be mistaken for
mastoiditis. They may also help to differentiate LST from
LS hypoplasia. They are due to mucosal edema and effusion,
likely secondary to venous congestion as a consequence of
LST. They all resolved during follow-up (MRI at 3
months).
The outcome of patients with isolated LST was better at
discharge than that of patients with other CVT, but, at 1 year,
there was no significant difference between the 2 groups.
Although the overall prognosis of isolated LST is good,
this condition cannot be considered benign because one
patient was still dependent at 1 year because of motor deficit
and dysphasia and another patient died during the acute
phase. Although at entry this patient who just presented
with headache and dysphasia had none of the recognized
factors of poor outcome,5 she rapidly became stuporous
because of worsening of intracerebral hemorrhage and
development of massive brain edema. Local thrombectomy
failed, decompressive craniectomy was performed, but she
died of pulmonary embolism. This case illustrates that, on
an individual basis, prognosis remains largely unpredictable and that, despite anticoagulation, pulmonary embolism remains a major cause of death in patients with CVT.
It also shows that, although the majority of patients with
isolated LST as well as with other CVT do well with
conventional treatment based on heparin, a few patients
require aggressive treatment such as decompressive surgery to prevent death from herniation.29,30
In summary, thrombosis limited to LS (with or without
involvement of tributary veins) accounts for one third of all
CVT in our series. Bilateral LST, without involvement of
other sinuses is rare, only one of 62.
Isolated LST does not differ from other CVT regarding
age, sex, and causes. In particular, it is today, at least in
adults, rarely secondary to an ear, nose, and throat infection.
Like in other CVT, the overall prognosis is good with 92%
of complete or nearly complete recovery at 1 year, but
management may eventually require decompressive surgery.
Pulmonary embolism remains a cause of death, even in
patients treated with heparin.
The most important finding in this series is that more than
two thirds of patients had headache as the only symptom
during the whole course of the disease. Only a minority of
such patients had papilledema so that altogether, isolated
headache was the clinical presentation in 45% of cases. This
stresses the need to systematically look for LST (and other
CVT) in patients with recent headache even in the absence of
associated symptoms and signs. Positive imaging of the
thrombosis itself together with the nonvisualization of the
sinus involved at MRI/MR angiography or CT/CT angiography are required to diagnose LST and rule out LS hypoplasia
and arachnoid granulations, which could be mistaken for
LST. Although some patients may recover spontaneously,1
heparin remains the treatment of choice in isolated LST as
well as in other varieties of CVT.2,31
Disclosures
None.
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Isolated Lateral Sinus Thrombosis: A Series of 62 Patients
Mariem Damak, Isabelle Crassard, Valérie Wolff and Marie-Germaine Bousser
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Stroke. 2009;40:476-481; originally published online December 31, 2008;
doi: 10.1161/STROKEAHA.107.509711
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