Cerebral Venous Thrombosis

Get CMECerebral Sinovenous ThrombosisThe patient complaint of “headache” often causes me to have a headache. There are so many things to ponder. We have previously covered some important conditions to consider when evaluating headache (ex, AVM, RMSF, Meningitis, Post-LP, Moyamoya, Migraine, Pseudotumor, and Pheochromocytoma). Many of these are rare and require our dedicated vigilance to ascertain the clues that point toward the diagnosis. Another entity that requires our super-sleuthing skills is Cerebral Venous Thrombosis (CVT).


CVT: Basics

  • CVT is rare, but has significant consequences [Saposnik, 2011]
    • Incidence is estimated to be ~0.6 per 100,000 children per year
    • Thrombosis of the venous system results in venous congestion and outflow obstruction creating increased capillary hydrostatic pressure. [Dlamini, 2010]
      • Increased hydrostatic pressure can lead to cerebral edema.
      • If pressures become high enough, can also compromise arterial flow and lead to ischemia.
      • Majority of infarcts become hemorrhagic.
    • Mortality is <10%, but persistent neurologic deficits can be seen in up to 79% of patients. [Dlamini, 2010]
  • Can affect all ages
    • ~40% of pediatric patients who have CVT are neonates
      • Increased thrombotic tendency in neonates. [Saposnik, 2011]
      • Mechanical forces on neonates head also increase risk. [Saposnik, 2011]
    • Non-neonates present similarly to adults [Star, 2013; deVeber, 2001]


CVT: Presentation

  • Clinical presentation is often subtle and not specific (once again, our job is challenging). [Dlamini, 2010]
  • Presentation varies with age:
    •  Neonates
      • Seizures
      • Lethargy
      • Respiratory Failure
      • Altered Mental State and Coma
    • Older infants and children present similarly to adults. [Star, 2013; deVeber, 2001]
      • Headache
      • Nausea / vomiting
      • Focal neurologic deficit / abnormality
        • Seizures
        • Hemiparesis
        • Papilledema
        • Ataxia
        • Speech or visual impairment
        • CN VI Palsy
  • Presentation can also vary based on location of thrombus. [Star, 2013]
    • Cerebral Venous Sinuses
      • Increased intracranial pressure
      • Headache, impaired vision, papilledema
    • Smaller Cerebral Veins
      • Focal venous ischemia
      • Focal neurologic deficits


CVT: Risk Factors

  • Risk factors often vary with age:
    • Neonates – perinatal complications, hypoxic encephalopathy, are most common
    • Younger children – head and neck infections are most common
    • Older children – chronic diseases are most common
  • Often CVT risk is multifactorial. [Dlamini, 2010Heller, 2003]
  • Local infection, injury, or abnormality
  • Systemic Illness
    • Dehydration
    • Sepsis
    • Cardiac Disease
    • Connective Tissue Disorders
    • Live Disease
    • Nephrotic Syndrome
    • Behcet’s Disease
    • Cancer
    • Anemia
  • Pro-thrombotic Disorders (noted in 12-50%)
    • Anticardiolipin antibody
    • Protein C and Protein S deficiencies
    • Factor V Leiden
    • Lupus Anticoagulant
    • Homocystinuria
  • Pro-coagulant Medications
    • Oral Contraceptive Pills
    • Asparaginase


CVT: Evaluation and Management

  • Imaging modality:
    • MRI with Venography is the modality of choice.
      • Occasionally, contrast is required given that thrombus can be difficult to distinguish from a hypoplastic sinus vein.
    • CT Venogram can also be used and is as effective as MRV, but has radiation (obviously).
  • Treatment: [Saposnik, 2011]
    • Rehydrate 
    • Treat associated infections
    • Treat seizures 
    • Treat increased intracranial pressure
    • Start anticoagulant
      • Treatment with anticoagulant has been deemed safe and effective. [Moharir, 2010]
        • Low Molecular Weight Heparin or Unfractionated Heparin are appropriate.
        • Oral Vitamin K Antagonist or LMWH will be continued for 3-6 months.
      • Recommended even in those who have hemorrhage noted at time of diagnosis. [Star, 2013Saposnik, 2011]
      • 31% of children who don’t receive anticoagulation have propagation of thrombus.


Moral of the Morsel

  • We see many patients with headaches… not all of them have benign causes. Be Vigilant!
    • Check for papilledema in cases presenting with headache.
    • Look for potential risk factors of CVT.
  • Thinking a patient has idiopathic intracranial hypertension (i.e., pseudotumor), check MRV when you get the MRI… there may be an associated Cerebral Venous Thrombosis!



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Sean M. Fox
Sean M. Fox
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