Cerebral Venous Thrombosis in Children

Gabriela de Toledo Passos Candelaria
Rio de Janeiro, Brazil
Leonardo R. Brandão
Sickkids, Toronto, Canada
Jorge David Aivazoglou Carneiro
Rio de Janeiro, Brazil

Summary

Discussion and Q&A Video

Rare condition in pediatric population: 0.34–0.67 cases/100.000/year

  • Affects primarily neonates
  • Results in neurologic impairment or death in approximately half the cases
  • Occurrence of venous infarcts or seizures portends a poor outcome
  • Increase in diagnostic frequency due to more sensitive and safe radiological exams
  • Greater clinical awareness of the condition necessary

Patient Cases

14-Year-Old Male Patient Admitted with Left Visual Loss and Altered Level of Consciousness

3-Year-Old Female Patient Admitted to ER with Fever and Vomiting

Takeaways

Predisposing Factor Is Often Present

  • Infections, dehydration, anemia, fever, hypoxic-ischemic injury
  • Head and neck infections (otitis media and mastoiditis, meningitis, sinusitis)
  • Head injury, post intracranial surgery
  • Heart disease, nephrotic syndrome, malignancy
  • Drugs (corticosteroids, L-asparaginase, oral contraceptives)

Clinical Features: Varying Symptoms

  • Seizures
  • Depressed level of consciousness and coma
  • Nausea and vomiting
  • Headache
  • Visual impairment
  • Neurological deficits
  • Neonates: seizures and diffuse neurologic signs

Diagnostic Imaging

  • Diagnosis: Lack of flow in the cerebral veins
  • Methods of choice for investigation: CT venography or MRI with venography

Treatment

Anticoagulation

  • Well tolerated by children and neonates (in the absence of any contraindication).
  • During the acute phase, anticoagulation is probably effective in reducing the risk of death and sequelae.
  • Anticoagulation is also effective in reducing the risk of recurrence.
  • Duration of anticoagulation needs to be individually tailored.
  • Prolonged treatment over 3-6 mo is justified according to individual factors.

Pretreatment intracranial hemorrhage: requires more careful consideration

  • Pathophysiology of hemorrhage in venous infarction involves venous/capillary hypertension and erythrocyte diapedesis or frank hemorrhage.
  • By preventing new thrombus formation, anticoagulation enables unopposed fibrinolysis to dissolve thrombi, relieving venous congestion.
  • Therefore, the potential ability of anticoagulant therapy to reduce intracranial hemorrhage caused by severe or persistent thrombosis may balance the risks of anticoagulant therapy dependent bleeding.

References

  1. deVeber G, Andrew M, Adams C, et al. Cerebral sinovenous thrombosis in children. N Engl J Med 2001;345:417-23.
  2. Barnes C, Newall F, Furmedge J, et al. Cerebral sinus venous thrombosis in children. J Pediatr Child Health 2004;40:53-5.
  3. Grunt S, Wingeier K,Wehrli E, et al. Cerebral sinus venous thrombosis in Swiss children. Dev Med Child Neurol 2010;52:1145-50.
  4. deVeber G, Chan A, Monagle P, et al. Anticoagulation Therapy in Pediatric Patients With Sinovenous Thrombosis. Arch Neurol 1998;55:1533-1537.
  5. Dlamini N, Billinghurst L, Kirkham FJ. Cerebral Venous Sinus (Sinovenous) Thrombosis in Children. Neurosurg Clin N Am 2010;21:511-527
  6. Guenther G, Arauz A. Cerebral venous thrombosis: A diagnostic and treatment update. Neurologia. 2011;26:488–98.
  7. Moharir M, Shroff M, Stephens D. Anticoagulants in Pediatric Cerebral Sinovenous Thrombosis. A Safety and Outcome Study. Ann Neurol 2010;67:590-599

 

Patient Case Video

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