cerebral venous thrombosis etiology, management and clinical features of various superficial and deep cebral venous thrombosis

1. Cerebral Venous and Sinus
Thrombosis
Presented By – Dr. Rahul Jain
Moderated by : Dr V.C. Jha
HoD Deptt of Neurosurgery, AIIMS Patna

2. Introduction
• Cerebral venous thrombosis (CVT) involves the dural
sinuses and/or cortical and deep cerebral veins.
• comprises 0.5–1% of strokes and usually affects young
people (78% occur in patients age < 50 years).
• incidence: 1.32 per 100,000 person years, with a higher
incidence of 2.78 in women 31–50 years of age.
• CVT affects all age groups and both sexes, but with a
strong preponderance in women between 20 and 40
years of age. This might reflect the fact that women in
that age group are more likely to use oral
contraceptives and to undergo puerperium.

3. Venous System of brain

5. Etiology

6. Others
• Vascular injury and compression owing to trauma or
mass lesions cause local endothelial damage and
altered hemodynamics.
• The association between dural arteriovenous fistulae
and CVT has been recognized, and it is thought to be
related to the aberrant rheology induced by the
fistulous connection.
• Infection is thought to cause CVT by altering the
coagulation cascade and inducing a hypercoagulable
state. Although infection has been a major cause of CVT
in the past, with the advent of modern antibiotic
therapy infectious CVT has become less common.

7. • Cavernous and transverse sinus thromboses are
most frequently associated with infections such as
sinusitis, otitis, and mastoiditis.
• Staphylococcus aureus is the most frequently
reported pathogen. In chronic forms, gram-
negative rods and fungi such as Aspergillus species
are most commonly isolated.
• dehydration and cachexia (marantic thrombosis):
includes burns and cachexia of neoplastic disease

8. Pathophysiology
• When thrombosis occurs in the cerebral veins and the
dural sinuses, the resulting venous hypertension
causes hypoxia of the brain, similar to the
symptomology of dural arteriovenous fistulae, with
resultant neuronal ischemia.
• Venous thrombosis likely also causes breakdown in the
blood-brain barrier, which leads to increased venous
pressure, increased intracranial blood volume, and
intracranial hypertension.
• The spectrum ranges from varying degrees of cerebral
edema to massive hemorrhage and bilateral cerebral
infarcts (Venous Infarction).

9. In Pregnancy/puerperium ?
• Highest risk is in the third trimenster and for 6–8
weeks post-partum.
• Incidence ≈ 1/10,000 births
• may be related to elevation of clotting factors (VII,
X, and especially factor VIII).
• Hypercoagulability increases post-partum due to
volume depletion and trauma.

10. Frequency of venous structures
involved
The relative frequency of involvement of dural sinuses
and other veins with thrombotic issues
1. sinuses
a) superior sagittal sinus (SSS) and left transverse sinus (TS)
(70% each)
b) multiple sinuses in 71%
c) isolated inferior sagittal sinus: rare, first case report in 1997
d) straight sinus
2. superficial cortical veins
3. deep venous system (e.g. internal cerebral vein)
4. cavernous sinus: rare.

11. Clinical Presentation
• no pathognomonic findings.
• signs and symptoms are due to elevated ICP as a result of
impaired venous outflow and may present as a syndrome
clinically indistinguishable from idiopathic intracranial
hypertension.
• Headache is the most common symptom. Nausea, vomiting,
and visual changes. Papilledema is seen in approximately
50% of patients, and confusion, agitation, and other mental
status changes occur in approximately 25% of patients.
• effect of cerebral venous thrombosis (CVT) on mental status
is quite variable, with some patients showing no change in
alertness, others developing mild confusion, and still others
progressing to coma.

12. Clinical presentation according to
the site and extent
1. Superior Sagittal Sinus
• anterior 1/3 of the SSS may occlude without neurologic
sequelae.
• Posterior to this (especially posterior to the vein of
Trolard), venous infarction is more likely to develop.
• Midportion SSS occlusion usually → increased muscle
tone ranging from spastic hemi- or quadriparesis to
decerebration.
• Posterior SSS thrombosis → field cuts or cortical
blindness, or massive stroke with cerebral edema and
death.

13. 2. Transverse Sinus
• Occlusion of the TS may occur without deficit
unless the contralateral TS is hypoplastic, in which
case presentation is similar to posterior SSS
occlusion.
• Transverse sinus CVT may be associated with
otalgia, otorrhea, cervical tenderness, and
lymphadenopathy from an underlying infection,
such as mastoiditis or otitis media.

14. 3. Cavernous Sinus Thrombosis
• eyelid edema, chemosis, retro-orbital pain, and
exophthalmos.
• Paralysis of cranial nerves III, IV, V1, V2, and VI can
occur because of their anatomic location within the
cavernous sinus leading to ophthalmoplegia and
decreased sensation in first division of trigeminal
nerve.
• Retinal hemorrhages and papilledema may be
present.

15. 4. Thrombosis in the jugular bulb may compress the
nerves in the jugular foramen pars nervosa causing
hoarseness, aphonia, difficulty swallowing and
breathlessness from involvement of cranial nerves IX,
X, XI, and XII with the jugular foramen syndrome
(vernet syndrome).
5. cortical vein thrombosis may be seen in the
absence of dural sinus involvement. It can present as
stroke syndrome. These cases are associated with
varied focal deficits, including aphasia, hemiparesis,
hemisensory loss, and hemianopia.

16. 6. When the thrombosis involves the deep venous
system, the patient can exhibit akinetic mutism,
coma, or decerebration. Mild cases causing memory
disturbances or minor confusion can occur.
7. Cerebellar vein thrombosis is extremely rare and
often lethal

17. Work up and Evaluation
• Diagnosis is made on the basis of clinical
presentation and imaging studies while clinical
laboratory studies are useful for determining the
possible causes of CVT.
• panel of routine blood studies including a complete
blood cell count, chemistry panel, and coagulation
factors.
• Guidelines for the diagnosis of CVT also
recommend a prothrombotic screen.

18. Lab studies
• CBC is performed to look for polycythemia. Decreased
platelet count would support thrombotic
thrombocytopenic purpura; leukocytosis might be seen
in sepsis.
• Antiphospholipid and anticardiolipin antibodies should
be obtained to evaluate for antiphospholipid syndrome.
• Sickle cell preparation or hemoglobin electrophoresis.
• ESR and antinuclear antibody studies should be
performed to screen for systemic lupus erythematosus,
Wegener granulomatosis, and temporal arteritis.

19. • Urine protein should be checked and, if elevated,
nephrotic syndrome considered. Liver function
studies should be performed to rule out cirrhosis.
• D-dimer values may be beneficial. In a study of 18
patients with cerebral venous thrombosis (CVT),
Tardy et al reported that D-dimer levels of less than
500 ng/mL had a negative predictive value of
99.6% for ruling out the diagnosis in patients with
acute headache.

20. Imaging
1. CT (30–100%
sensitivity & 83–100%
specificity)
• Venous infarct.
• hyperdense sinus
(NCCT)
• Dense delta sign (NCCT)
• empty delta sign (CECT)

21. 2. MRI
• modality of choice
• MRA/MRV is now the
best method
• shows absence of flow
and clot burden and
demonstrates
parenchymal changes
including venous
infarcts.

22. 3. Cerebral angiography
• now only indicated when
MRI diagnosis is uncertain
or when intervention is
desired.
• finding is nonvisualization
of all or part of a sinus
during the venous phase.
• In cortical vein thrombosis,
thereis a sudden stop of the
occluded vein, which may
be surrounded by dilated
collateral “corkscrew
vessels.

23. 4. SSPCA
• Single-slice phase-contrast angiography (SSPCA)
takes less than 30 seconds and provides rapid and
reliable information.
• Many neurologists now consider it to be the
procedure of choice in diagnosing cerebral venous
thrombosis
• In a study of 21 patients, Adams demonstrated a
specificity and sensitivity of 100% for SSPCA when
compared with alternative imaging techniques.

24. Treatment
• Driving principle: treat the underlying abnormality.
• Management should be aggressive because
recoverability of brain is probably greater than with
arterial occlusive stroke.
• Management is challenging because measures that
counteract thrombosis (e.g. anticoagulation) tend
to increase the risk of hemorrhagic infarct (the risk
of which is already increased), and measures that
lower ICP tend to increase blood viscosity →
increased coagulability.

25. • Guidelines devised by 2011 AHA Scientific
Statement for the Diagnosis and Management of
Cerebral Venous Thrombosis and the 2018 Report
of the Society of NeuroInterventional Surgery on
Endovascular Strategies for Cerebral Venous
Thrombosis.

26. 1. general measures
a) patients suspected of having infection should receive appropriate
treatment: antibiotics, drainage of purulent collection. (Level I)
b) seizures:
● AED for a defined duration are recommended for a single seizure
with (Level I) or without (Level II) parenchymal lesions to prevent
futher seizures
● AEDs are not recommended in the absence of seizures (potential
harm)
c) steroids: not recommended unless needed for other underlying
disease (potential harm) (reduces fibrinolysis, increases
coagulation)
d) control HTN

27. 2. anticoagulation
a) anticoagulation: start with with dose-adjusted
unfractionated heparin (UFW) or weightbased LMWH,
(Level II). It remains the best treatment even when there
is evidence of intracerebral hemorrhage (ICH) with the
attendant risk of increasing the size of the hemorrhage.
There is no consensus on duration of treatment. Success
rate may be higher if administered before patient
becomes moribund.
★ during pregnancy, full dose LMWH is recommended
over UFH (Level II)
b) transition from heparin to vitamin K antagonists (e.g.
warfarin)

28. 3. Monitor ICP if patient continues to deteriorate: ventriculostomy is
preferred, but use caution in placing catheter if patient is on heparin
a) hydrate aggressively as ICP tolerates
b) measures to lower ICP: differs somewhat from treatment for traumatic ICP
elevation
● acetazolamide (Level II): one of the few measure to treat elevated ICP
that does not rely on venous outflow
● elevate HOB
● drain CSF
● pentobarbital coma: also does not depend on venous outflow
● hyperosmotic and/or loop diuretics: reserve for last because diuretics
→ hypertonicity →↑ viscosity →↑ coagulation. Replace fluid loss with
isotonic IV fluids to prevent dehydration; i.e., goal is hypertonic
euvolemia
c) monitor patients with increased ICP for progressive visual loss and treat
elevated ICP urgently if detected (Level I). Treatment measures include: serial
LPs, optic nerve sheath fenestration, or VP shunt (Level II)

29. 4. Endovascular therapy (EVT) for deterioration
despite intensive anticoagulation (Level II). There are
no guidelines for how long towait before declaring
medical therapy a failure, decision takes into account
how sick the patient is. Also, there is no information
when to use Decompressive Craniectomy vs. EVT.
Modalities include chemical thrombolysis (direct
injection of tPA into the sinus) and mechanical
thrombectomy.

30. 5. Decompressive craniectomy (DC):
• consider for neurologic deterioration due to mass
effect or intracranial hemorrhage causing increased
ICP(Level II).
• Early surgery (≤ 12 hrs from admission) & younger
patients are predictors for a more favorable
response to DC. If DC is elected, the options of
heparinization and tPA cannot be employed for ≈
2–3 days.

31. 6. Long-term anticoagulation after resolution of acute
phase with heparin (Level II):
a) spontaneous CVT: vitamin K antagonists (VKA) for 6–12
months, target INR = 2–3
b) CVT with risk factors that have been eliminated: VKA
for 3–6 months, target INR = 2–3
c) recurrent CVT, VTE after CVT, or severe thrombophilia
(e.g. homozygous prothrombin G20210A, homozygous
factor V Leiden, protein C or S or antithrombin deficiency,
antiphospholipid syndrome, or combined thrombophilic
conditions): indefinite VKA, target INR = 2–3
d) consider consultation with a physician with expertise
in coagulation
e) for CVT during pregnancy: continue full
anticoagulation with LMWH during the pregnancy, and
LMWH or VKA with a target INR = 2–3 for ≥ 6 weeks post-
partum (for a total duration of therapy of 6 months)
(Level I)

32. Prognosis
• Natural history (without treatment): reported mortality range is
14–40%
• With anticoagulation: ≈ 13% die or remain dependent.
• Poor prognosticators:
1. clinical status:
a) coma
b) rapid neurologic deterioration, focal signs
2. demographics
a) age: extremes of age (infancy or elderly)
b) male gender
3. radiographic findings:
a) hemorrhages, especially larger hemorrhages
b) venous infarcts
4. deep venous involvement

33. • Herniation attributable to unilateral mass effect is
the major cause of death in CVT.
• Smith demonstrated the efficacy of anticoagulant
and thrombolytic therapy in patients with cerebral
venous thrombosis (CVT).

34. Future pregnancies in patients with a history of CVT???
1. advise the patient that future pregnancy is not
contraindicated. Consultation with a haematologist
and/or maternal fetal medicine specialist is reasonable.
(Level II)
2. prophylaxis with LMWH during future pregnancies
and the post-partum period for women with a history of
CVT is recommended. (Level II)

35. Conclusion
• CVT is a relatively rare entity, with a wide spectrum
of clinical presentation, natural history, and
outcomes.
• A high level of suspicion is necessary for clinicians
to make an early diagnosis, although recent
advances in diagnostic and therapeutic modalities
have changed the detection and prognosis of this
disease.

36. • The rarity of this disease will confound attempts to
create rigorous, evidence-based approaches to
care, but guidelines from the AHA and European
Federation of Neurological Societies provide a
framework through which to approach diagnosis,
management, and expected outcomes for patients
with CVT.

37. References
1. Greenberg’s Handbook of neurosurgery 9th Ed.
2. Youmann’s and Winn Neurological surgery 7th Ed.
3. Medscape Consult.

38. THANK YOU