Cerebral Venous Thrombosis

Cerebral 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!

 

References

Carducci C1, Colafati GS2, Figà-Talamanca L2, Longo D2, Lunardi T2, Randisi F2, Bernardi B2. Cerebral sinovenous thrombosis (CSVT) in children: what the pediatric radiologists need to know. Radiol Med. 2016 May;121(5):329-41. PMID: 27025499. [PubMed] [Read by QxMD]

Cerebral sinovenous thrombosis (CSVT) is a relatively uncommon and potentially life-threatening condition in childhood, occurring in various clinical settings. Nowadays, however, it is increasingly diagnosed as related to many causes, likely due to greater clinical awareness and improvement of neuroradiologic techniques. The prompt diagnosis is an important goal to significantly reduce the risk of acute complications and long-term sequelae. The p […]

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Zuurbier SM1, Middeldorp S2, Stam J3, Coutinho JM3. Sex differences in cerebral venous thrombosis: A systematic analysis of a shift over time. Int J Stroke. 2016 Feb;11(2):164-70. PMID: 26783307. [PubMed] [Read by QxMD]

In contemporary studies, cerebral venous thrombosis is three times more common in adult women than in men. […]

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Selvitop O1, Poretti A1, Huisman TA1, Wagner MW2. Cerebral sinovenous thrombosis in a child with Crohn’s disease, otitis media, and meningitis. Neuroradiol J. 2015 Jun;28(3):274-7. PMID: 26246095. [PubMed] [Read by QxMD]

Pediatric cerebral sinovenous thrombosis (CSVT) is associated with high morbidity and mortality. Severe long-term sequelae are reported in up to 48% of children. The most frequent location of CSVT in children is the superficial venous system. We present the neuroimaging findings using both computed tomography and magnetic resonance imaging (MRI) in a 10-year-old child with extensive superficial CSVT. Our report aims to stress the importance of aw […]

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Star M1, Flaster M. Advances and controversies in the management of cerebral venous thrombosis. Neurol Clin. 2013 Aug;31(3):765-83. PMID: 23896504. [PubMed] [Read by QxMD]

Cerebral venous thrombosis (CVT) is a rare form of stroke found most often in young women of reproductive age, often associated with oral contraceptive use, genetic or acquired thrombophilia, pregnancy, dehydration, or infection. CVT should be considered in any young patient who presents with an unexplained headache in combination with known hypercoagulable state, focal neurologic deficits, seizure, lobar hemorrhage, or bilateral thalamic or basa […]

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Saposnik G, Barinagarrementeria F, Brown RD Jr, Bushnell CD, Cucchiara B, Cushman M, deVeber G, Ferro JM, Tsai FY; American Heart Association Stroke Council and the Council on Epidemiology and Prevention. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011 Apr;42(4):1158-92. PMID: 21293023. [PubMed] [Read by QxMD]

The purpose of this statement is to provide an overview of cerebral venous sinus thrombosis and to provide recommendations for its diagnosis, management, and treatment. The intended audience is physicians and other healthcare providers who are responsible for the diagnosis and management of patients with cerebral venous sinus thrombosis. […]

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Moharir MD1, Shroff M, Stephens D, Pontigon AM, Chan A, MacGregor D, Mikulis D, Adams M, deVeber G. Anticoagulants in pediatric cerebral sinovenous thrombosis: a safety and outcome study. Ann Neurol. 2010 May;67(5):590-9. PMID: 20437556. [PubMed] [Read by QxMD]

Clinical trials are lacking in pediatric cerebral sinovenous thrombosis (CSVT). Neonates and children increasingly receive anticoagulant therapy (ACT) based on adult studies. Safety data for ACT in pediatric CSVT are scant and urgently needed. The objective was to assess the safety and outcome of ACT in pediatric CSVT. […]

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Dlamini N1, Billinghurst L, Kirkham FJ. Cerebral venous sinus (sinovenous) thrombosis in children. Neurosurg Clin N Am. 2010 Jul;21(3):511-27. PMID: 20561500. [PubMed] [Read by QxMD]

Cerebral venous sinus (sinovenous) thrombosis (CSVT) in childhood is a rare, but underrecognized, disorder, typically of multifactorial etiology, with neurologic sequelae apparent in up to 40% of survivors and mortality approaching 10%. There is an expanding spectrum of perinatal brain injury associated with neonatal CSVT. Although there is considerable overlap in risk factors for CSVT in neonates and older infants and children, specific differen […]

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Stienen A1, Weinzierl M, Ludolph A, Tibussek D, Häusler M. Obstruction of cerebral venous sinus secondary to idiopathic intracranial hypertension. Eur J Neurol. 2008 Dec;15(12):1416-8. PMID: 19049565. [PubMed] [Read by QxMD]

Whether cerebral venous sinus obstruction is a cause or consequence of idiopathic intracranial hypertension (IIH) is uncertain. […]

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Heller C1, Heinecke A, Junker R, Knöfler R, Kosch A, Kurnik K, Schobess R, von Eckardstein A, Sträter R, Zieger B, Nowak-Göttl U; Childhood Stroke Study Group. Cerebral venous thrombosis in children: a multifactorial origin. Circulation. 2003 Sep 16;108(11):1362-7. PMID: 12939214. [PubMed] [Read by QxMD]

The present study was performed to assess the association of prothrombotic risk factors and underlying conditions (infections, vascular trauma, immobilization, malignancies, autoimmune diseases, renal diseases, metabolic disorders, obesity, birth asphyxia, cardiac malformations, and use of prothrombotic drugs) with cerebral venous thrombosis (CVT) in children. […]

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Carvalho KS1, Garg BP. Cerebral venous thrombosis and venous malformations in children. Neurol Clin. 2002 Nov;20(4):1061-77. PMID: 12616681. [PubMed] [Read by QxMD]

Venous strokes are not as common as arterial strokes in the pediatric population, but may be associated with significant mortality and morbidity. Cerebral vein thrombosis and venous sinus thrombosis are responsible for most venous strokes. Vein of Galen malformation is a rare but important cause of mortality in neonates and infants. Awareness of these potential causes of stroke in the pediatric population, early diagnosis, and appropriate therape […]

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deVeber G1, Andrew M, Adams C, Bjornson B, Booth F, Buckley DJ, Camfield CS, David M, Humphreys P, Langevin P, MacDonald EA, Gillett J, Meaney B, Shevell M, Sinclair DB, Yager J; Canadian Pediatric Ischemic Stroke Study Group. Cerebral sinovenous thrombosis in children. N Engl J Med. 2001 Aug 9;345(6):417-23. PMID: 11496852. [PubMed] [Read by QxMD]

Cerebral sinovenous thrombosis in children is a serious disorder, and information is needed about its prevention and treatment. […]

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