Skip to main content
Log in

Treatment and prevention of cerebrovascular disorders in children

  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Opinion statement

Cerebrovascular disorders are an important cause of mortality and chronic morbidity in children. Ischemic stroke is more common than cerebral venous thrombosis and hemorrhagic stroke in children. Several medical disorders have been associated with stroke in children, and a thorough evaluation of underlying causes is needed to determine the best treatment and prevention strategy. The treatment and prevention of stroke in children is not well studied, and current recommendations are based on adult studies, nonrandomized trials, or expert opinion. Children with stroke require immediate, special attention and if possible should be stabilized and transferred to an institution that can offer pediatric neurovascular expertise and care. All children with stroke should be referred to or have their care managed by a pediatric neurologist. The treatment of stroke in adults is well studied, and when applicable this evidence should be considered in the treatment of children with stroke. Data from animal and adult stroke studies have demonstrated a benefit for the aggressive treatment of infection, fever, blood pressure, hypo/hyperglycemia, intracranial pressure, and seizures, and should be applied to children with stroke. The use of thrombolytic, antithrombotic, and antiplatelet therapies is based on adult studies, cohort studies, and/or expert opinion. Two consensus guidelines regarding the treatment of arterial ischemic stroke and cerebral venous thrombosis were recently published and recommend the use of anticoagulants or antiplatelet agents in the acute setting, depending on the underlying cause of stroke. The evidence for the primary prevention of stroke in children is restricted to sickle cell disease (SCD) and derived from the Stroke Prevention in Sickle Cell Study Project studies. Long-term chronic transfusion therapy to maintain hemoglobin S levels below 30% is indicated in children with SCD and intracranial stenosis. It has also been recently determined that chronic transfusion therapy should not be stopped in children with SCD and an increased risk for stroke. The recurrence rate of arterial ischemic stroke (AIS) in children ranges from 6% to 30% and is highest among children with recurrent transient ischemic attack, cardiac disease, arteriopathies, and metabolic and coagulation abnormalities. Recommendations for secondary prevention are based on adult studies and the underlying pathophysiology of the stroke. Antiplatelet therapy (aspirin 1–5 mg/ kg/day) is recommended in most children with a history of AIS. Although there is minimal evidence to support its use in children, anticoagulation may be indicated in AIS associated with extracranial arterial dissection, prothrombotic disorders, cardiac disease, severe intracranial stenosis, and recurrent AIS while on antiplatelet therapy.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

Referenees and Reeommended Reading

  1. Sehoenberg BS, Mellinger JF, Sehoenberg DG: Cerebrovaseular disease in infants and ehildren: a study of ineidenee, elinieal features, and survival. Neurology 1978, 28: 763–768.

    Google Scholar 

  2. Earley CJ, Kittner SJ, Feeser BR, et al.: Stroke in ehildren and siekle-eell disease: Baltimore-Washington Cooperative Young Stroke Study. Neurology 1998, 51: 169–176.

    PubMed  CAS  Google Scholar 

  3. Giroud M, Lemesle M, Gouyon JB, et al.: Cerebrovaseular disease in ehildren under 16 years of age in the eity of Dijon, Franee: a study of ineidenee and elinieal features from 1985 to 1993. / Clin Epidemiol 1995, 48: 1343–1348.

    Article  CAS  Google Scholar 

  4. Zahuranee DB, Brown DL, Lisabeth LD, Morgenstern LB: Is it time for a large, eollaborative study of pediatric stroke? Stroke 2005, 36: 1825–1829.

    Article  Google Scholar 

  5. Paediatric Stroke Working Group. Stroke in ehildhood: elinieal guidelines for diagnosis, management and rehabilitation. London: Royal College of Physieians of London; 2004. This publieation represents evidenee-based eonsensus guidelines on pediatric stroke from the Clinieal Effeetiveness and Evaluation Unit of the Royal College of Physieians. The guidelines were developed by a pediatric stroke working group that eonsisted of experts in eerebrovaseular disease, pediatric neurology, pediatrics, neuroradiology, hematology, psyehology, and rehabilitation medieine. The guidelines are based on a thorough review and elassifieation of epidemiologie evidence, and reeommendations are provided according to universally accepted criteria.

  6. Monagle P, Chan A, Massieotte P, et al.: Antithrombotic therapy in ehildren: the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy. Chest 2004, 126: 645S-687S. These evidence-based eonsensus guidelines on pediatric stroke are derived from the Seventh American College of Chest Physicians on Antithrombotic and Thrombolytic Therapy. The guidelines were developed by a group of pediatric hematologists. The guidelines are based on a thorough review and elassifieation of epidemiologie evidence, and recommendations are provided according to universally accepted criteria.

    Article  PubMed  CAS  Google Scholar 

  7. Ganesan V, Prengler M, McShane MA et al.: Investigation of risk faetors in ehildren with arterial isehemic stroke. Ann Neurol 2003, 53: 167–173.

    Article  PubMed  Google Scholar 

  8. Kumar K: Neurologieal complieations of congenital heart disease. Indian J Pediatr 2000, 67: S15-S19.

    PubMed  CAS  Google Scholar 

  9. Chabrier S, Husson B, Lasjaunias P, et al.: Stroke in childhood: outcome and recurrence risk by mechanism in 59 patients. J Child Neurol 2000, 15: 290–294.

    PubMed  CAS  Google Scholar 

  10. Sebire G, Fullerton H, Riou E, deVeber G: Toward the definition of cerebral arteriopathies of childhood. Curr Opin Pediatr 2004, 16: 617–622.

    Article  PubMed  Google Scholar 

  11. Fullerton HJ, Johnston SC, Smith WS: Arterial dissection and stroke in children. Neurology 2001, 57: 1155–1160.

    PubMed  CAS  Google Scholar 

  12. MeKusiek VA: Online Mendelian inheritance in man. OMIM 2004.

  13. Horn P, Pfister S, Bueltmann E, et al.: Moyamoya-like vaseulopathy (moyamoya syndrome) in children. Childs Nerv Syst 2004, 20: 382–391.

    Article  PubMed  Google Scholar 

  14. Seothorn DJ, Priee C, Sehwartz D, et al.: Risk of recurrent stroke in ehildren with siekle eell disease reeeiving blood transfusion therapy for at least five years after initial stroke. J Pediatr 2002, 140: 348–354.

    Article  Google Scholar 

  15. Powars D, Wilson B, Imbus C, et al.: The natural history of stroke in siekle cell disease. Am J Med 1978, 65: 461–471.

    Article  PubMed  CAS  Google Scholar 

  16. Buehanan GR, DeBaun MR, Quinn CT, Steinberg MH: Siekle eell disease. Hematology 2004, 9: 35–47.

    Article  Google Scholar 

  17. Hoppe C, Klitz W, Cheng S, et al.: Gene interaetions and stroke risk in ehildren with siekle eell anemia. Blood 2004, 103: 2391–2396.

    Article  PubMed  CAS  Google Scholar 

  18. Adams RJ: Stroke prevention and treatment in siekle eell disease. Areh Neurol 2001, 58: 565–568.

    Article  CAS  Google Scholar 

  19. Kirkham FJ, Prengler M, Hewes DK, Ganesan V: Risk factors for arterial isehemic stroke in ehildren. J Child Neurol 2000, 15: 299–307.

    PubMed  CAS  Google Scholar 

  20. Steen RG, Emudianughe T, Hankins GM, et al.: Brain imaging findings in pediatric patients with siekle cell disease. Radiology 2003, 228: 216–225.

    Article  PubMed  Google Scholar 

  21. Akar N, Akar E, Deda G, et al.: Faetor V1691 G-A, prothrombin 20210 G-A, and methylenetetrahydrofolate reduetase 677 C-T variants in Turkish children with cerebral infarct. J Child Neurol 1999, 14: 749–751.

    PubMed  CAS  Google Scholar 

  22. Kenet G, Sadetzki S, Murad H, et al.: Faetor V Leiden and antiphospholipid antibodies are significant risk factors for isehemic stroke in children. Stroke 2000, 31: 1283–1288.

    PubMed  CAS  Google Scholar 

  23. Nowak-Gottl U, Strater R, Heineeke A, et al.: Lipoprotein (a) and genetie polymorphisms of dotting faetor V, prothrombin, and methylenetetrahydrofolate reduetase are risk faetors of spontaneous isehemic stroke in childhood. Blood 1999, 94: 3678–3682.

    PubMed  CAS  Google Scholar 

  24. Hagstrom JN, Walter J, Bluebond-Langner R, et al.: Prevalence of the factor V Leiden mutation in ehildren and neonates with thromboembolie disease. J Pediatr 1998, 133: 777–781.

    Article  PubMed  CAS  Google Scholar 

  25. Bonduel M, Seiueeati G, Hepner M, et al.: Factor V Leiden and prothrombin gene G20210A mutation in children with cerebral thromboembolism. Am J Hematol 2003, 73: 81–86.

    Article  PubMed  CAS  Google Scholar 

  26. McColl MD, Chalmers EA Thomas A et al.: Factor V Leiden, prothrombin 20210G->A and the MTHFR C677T mutations in childhood stroke. Thromb Haemost 1999, 81: 690–694.

    PubMed  CAS  Google Scholar 

  27. Zenz W, Bodo Z, Plotho J, et al.: Factor V Leiden and prothrombin gene G 20210 A variant in children with isehemic stroke. Thromb Haemost 1998, 80: 763–766.

    PubMed  CAS  Google Scholar 

  28. Strater R, Becker S, von Eckardstein A, et al.: Prospective assessment of risk factors for recurrent stroke during childhood--a 5-year follow-up study. Laneet 2002, 360: 1540–1545.

    Google Scholar 

  29. Lanthier S, Cannant L, David M, et al.: Stroke in children: the coexistence of multiple risk factors predicts poor outcome. Neurology 2000, 54: 371–378.

    PubMed  CAS  Google Scholar 

  30. Lindsberg PJ, Grau AJ: Inflammation and infections as risk factors for ischemic stroke. Stroke 2003, 34: 2518–2532.

    Article  PubMed  Google Scholar 

  31. Snyder RD, Stowing J, Cushing AH, et al.: Cerebral infarction in childhood bacterial meningitis. / Neurol Neurosurg Psychiatr 1981, 44: 581–585.

    CAS  Google Scholar 

  32. Narayan P, Samuels OB, Barrow DL: Stroke and pediatric human immunodeficiency virus infection. Case report and review of the literature. Pediatr Neurosurg 2002, 37: 158–163.

    Article  PubMed  Google Scholar 

  33. Guidi B, Bergonzini P, Crisi G, et al.: Case of stroke in a 7-year-old male after parvovirus B19 infection. Pediatr Neurol 2003, 28: 69–71.

    Article  PubMed  Google Scholar 

  34. Bell ML, Buchhalter JR: Influenza A-associated stroke in a 4-year-old male. Pediatr Neurol 2004, 31: 56–58.

    Article  PubMed  Google Scholar 

  35. Sebire G, Meyer L, Chabrier S: Varicella as a risk factor for cerebral infarction in childhood: a case-control study. Ann Neurol 1999, 45: 679–680.

    Article  PubMed  CAS  Google Scholar 

  36. Ichiyama T, Houdou S, Kisa T, et al.: Varicella with delayed hemiplegia. Pediatr Neurol 1990, 6: 279–281.

    Article  PubMed  CAS  Google Scholar 

  37. Askalan R, Laughlin S, Mayank S, et al.: Chickenpox and stroke in childhood: a study of frequency and causation. Stroke 2001, 32: 1257–1262.

    PubMed  CAS  Google Scholar 

  38. Lynch JK: Cerebrovascular disorders in children. Curr Neurol Neurosci Rep 2004, 4: 129–138.

    Article  PubMed  Google Scholar 

  39. Barnes C, deVeber G: Prothrombotic abnormalities in childhood ischaemic stroke. Thromb Res 2005, In press.

  40. deVeber G, Monagle P, Chan A, et al.: Prothrombotic disorders in infants and children with cerebral throm-boembolism. Arch Neurol 1998, 55: 1539–1543.

    Article  PubMed  CAS  Google Scholar 

  41. Heller C, Heinecke A, Junker R, et al.: Cerebral venous thrombosis in children: a multifactorial origin. Circulation 2003, 108: 1362–1367.

    Article  PubMed  Google Scholar 

  42. Muthukumar N: Uncommon cause of sinus thrombosis following closed mild head injury in a child. Childs Nerv Syst 2004, 21: 86–88.

    Article  PubMed  Google Scholar 

  43. Sousa J, O’Brien D, Bartlett R, Vaz J: Sigmoid sinus thrombosis in a child after dosed head injury. Br J Neurosurg 2004, 18: 187–188.

    Article  PubMed  CAS  Google Scholar 

  44. Stiefel D, Eich G, Sacher P: Posttraumatic durai sinus thrombosis in children. Eur] Pediatr Surg 2000, 10: 41–44.

    CAS  Google Scholar 

  45. Huisman TA, Holzmann D, Martin E, Willi UV: Cerebral venous thrombosis in childhood. Eur Radiol 2001, 11: 1760–1765.

    Article  PubMed  CAS  Google Scholar 

  46. Al Jarallah A Al Rifai MT, Riela AR, Roach ES: Nontraumatic brain hemorrhage in children: etiology and presentation. J Child Neurol 2000, 15: 284–289.

    PubMed  CAS  Google Scholar 

  47. Di Rocco C, Tamburrini G, Rollo M: Cerebral arteriovenous malformations in children. Acta Neurochir (Wien) 2000, 142: 145–156.

    Article  Google Scholar 

  48. Eeg-Olofsson O, Ringheim Y: Stroke in children. Clinical characteristics and prognosis. Acta Paediatr Scand 1983, 72: 391–395.

    PubMed  CAS  Google Scholar 

  49. Punt J: Surgical management of paediatric stroke. Pediatr Radiol 2004, 34: 16–23.

    Article  PubMed  Google Scholar 

  50. Meyer-Heim AD, Boltshauser E: Spontaneous intracranial haemorrhage in children: aetiology, presentation and outcome. Brain Dev 2003, 25: 416–421.

    Article  PubMed  Google Scholar 

  51. Chung B, Wong V: Pediatric stroke among Hong Kong Chinese subjects. Pediatrics 2004, 114: e206-e212.

    Article  PubMed  Google Scholar 

  52. Butros LJ, Bussel JB: Intracranial hemorrhage in immune thrombocytopenic purpura: a retrospective analysis. / Pediatr Hematol Oncol 2003, 25: 660–664.

    Article  Google Scholar 

  53. Laurent JP, Bruce DA Schut L: Hemorrhagic brain tumors in pediatric patients. Childs Brain 1981, 8: 263–270.

    Article  PubMed  CAS  Google Scholar 

  54. Broderick J, Talbot GT, Prenger E, et al.: Stroke in children within a major metropolitan area: the surprising importance of intracerebral hemorrhage. J Child Neu-rol 1993, 8: 250–255.

    Article  CAS  Google Scholar 

  55. Roach ES, Riela AR: Pediatric Cerebrovascular Disorders, edn 2. Armonk, NY: Futura Publishing Company; 1995.

    Google Scholar 

  56. Hutchison JS, Ichord R, Guerguerian AM, deVeber G: Cerebrovascular disorders. Semin Pediatr Neurol 2004, 11: 139–146.

    Article  PubMed  Google Scholar 

  57. Adelson PD, Bratton SL, Carney NA et al.: Guidelines for the acute medical management of severe traumatic brain injury in infants, children, and adolescents. Chapter 8. Cerebral perfusion pressure. Pediatr Crit Care Med 2003, 4: S31-S33.

    Article  PubMed  Google Scholar 

  58. Capes SE, Hunt D, Malmberg K, et al.: Stress hyperglycemia and prognosis of stroke in nondiabetic and diabetic patients: a systematic overview. Stroke 2001, 32: 2426–2432.

    PubMed  CAS  Google Scholar 

  59. Wirrell EC, Armstrong EA, Osman LD, Yager JY: Prolonged seizures exacerbate perinatal hypoxic-ischemic brain damage. Pediatr Res 2001, 50: 445–454.

    Article  PubMed  CAS  Google Scholar 

  60. Thirumalai SS, Shubin RA: Successful treatment for stroke in a child using recombinant tissue plasminogen activator. J Child Neurol 2000, 15: 558.

    PubMed  CAS  Google Scholar 

  61. Noser EA, Felberg RA, Alexandrov AV: Thrombolytic therapy in an adolescent ischemic stroke. Child Neurol 2001, 16: 286–288.

    CAS  Google Scholar 

  62. Golomb MR, Rafay M, Armstrong D, et al.: Intra-arterial tissue plasminogen activator for thrombosis complicating cerebral angiography in a 17-year-old girl. / Child Neurol 2003, 18: 420–423.

    Google Scholar 

  63. Younkin DP: Diagnosis and treatment of ischemic pediatrie stroke. Curr Neurol Neurosci Rep 2002, 2: 18–24.

    Article  PubMed  Google Scholar 

  64. Wang M, Hays T, Balasa V, et al.: Low-dose tissue plasminogen activator thrombolysis in children. / Pediatr Hematol Oncol 2003, 25: 379–386.

    Article  Google Scholar 

  65. AdamsHP Jr, Adams RJ, Brott T, et al.: Guidelines for the early management of patients with ischemic stroke: a scientific statement from the Stroke Council of the American Stroke Association. Stroke 2003, 34: 1056–1083. This publication represents evidence-based consensus guidelines on adult ischemie stroke from the American Stroke Association. The guidelines were developed by the Stroke Council, which consists of experts in the area of cerebrovascular disease. The guidelines are based on a thorough review and classification of epidemiologic evidence, and recommendations are provided according to universally accepted criteria.

    Article  PubMed  Google Scholar 

  66. Leaker M, Massicotte MP, Brooker LA Andrew M: Thrombolytic therapy in pediatric patients: a comprehensive review of the literature. Thromb Haemost 1996, 76: 132–134.

    PubMed  CAS  Google Scholar 

  67. Gabis LV, Yangala R, Lenn NJ: Time lag to diagnosis of stroke in children. Pediatrics 2002, 110: 924–928.

    Article  PubMed  Google Scholar 

  68. Andrew M, Marzinotto V, Massicotte P, et al.: Heparin therapy in pediatric patients: a prospective cohort study. Pediatr Res 1994, 35: 78–83.

    Article  PubMed  CAS  Google Scholar 

  69. Massicotte P, Adams M, Marzinotto V, et al.: Low-molecular-weight heparin in pediatric patients with thrombotic disease: a dose finding study. / Pediatr 1996, 128: 313–318.

    Article  CAS  Google Scholar 

  70. Strater R, Kurnik K, Heller C, et al.: Aspirin versus lowdose low-molecular-weight heparin: antithrombotic therapy in pediatric ischemie stroke patients: a prospective follow-up study. Stroke 2001, 32: 2554–2558.

    PubMed  CAS  Google Scholar 

  71. Hune S, Rafay MF, Domi T, et al.: Plavix (clopidogrel) in pediatric stroke: monitoring of side effects and patient education strategies [abstract]. Stroke 2004, 35: 284.

    Google Scholar 

  72. Lee MC, Frank JI, Kahana M, et al.: Decompressive hemicraniectomy in a 6-year-old male after unilateral hemispheric stroke. Case report and review. Pediatr Neurosurg 2003, 38: 181–185.

    Article  PubMed  Google Scholar 

  73. deVeber G, Chan A, Monagle P, et al.: Anticoagulation therapy in pediatric patients with sinovenous thrombosis: a cohort study. Arch Neurol 1998, 55: 1533–1537.

    Article  PubMed  CAS  Google Scholar 

  74. Dix D, Andrew M, Marzinotto V, et al.: The use of low molecular weight heparin in pediatric patients: a prospective cohort study. / Pediatr 2000, 136: 439–445.

    Article  CAS  Google Scholar 

  75. Streif W, Mitchell LG, Andrew M: Antithrombotic therapy in children. Curr Opin Pediatr 1999, 11: 56–64.

    Article  PubMed  CAS  Google Scholar 

  76. deVeber G, Andrew M, Canadian Pediatric Ischemic Stroke Study Group: Cerebral sinovenous thrombosis in children. N Engl J Med 2001, 345: 417–423.

    Article  PubMed  CAS  Google Scholar 

  77. Shroff M, deVeber G: Sinovenous thrombosis in children. Neuroimaging Clin N Am 2003, 13: 115–138.

    Article  PubMed  Google Scholar 

  78. Canhao P, Falcao F, Ferro JM: Thrombolytics for cerebral sinus thrombosis: a systematic review. Cerebrovasc Dis 2003, 15: 159–166.

    Article  PubMed  CAS  Google Scholar 

  79. Broderick JP, Adams HP Jr, Barsan W, et al.: Guidelines for the management of spontaneous intracerebral hemorrhage: a statement for healthcare professionals from a special writing group of the Stroke Council, American Heart Association. Stroke 1999, 30: 905–915.

    PubMed  CAS  Google Scholar 

  80. Smith ER, Butler WE, Ogilvy CS: Surgical approaches to vascular anomalies of the child’s brain. Curr Opin Neurol 2002, 15: 165–171.

    Article  PubMed  Google Scholar 

  81. Adams RJ, McKie VC, Hsu L, et al.: Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl f Med 1998, 339: 5–11.

    Article  CAS  Google Scholar 

  82. NHLBI Communications Office: NHLBI Stops Study Testing How Long Children with Sickle Cell Anemia Should Have Blood Transfusions to Prevent Stroke. Washington, DC: 2004.

  83. Clancy RR, McGaurn SA, Goin JE, et al.: Allopurinol neurocardiac protection trial in infants undergoing heart surgery using deep hypothermie circulatory arrest. Pediatrics 2001, 108: 61–70.

    Article  PubMed  CAS  Google Scholar 

  84. Houkin K, Kuroda S, Nakayama N: Cerebral revascularization for moyamoya disease in children. Neurosurg Clin N Am 2001, 12: 575–584, ix.

    PubMed  CAS  Google Scholar 

  85. Fung LW, Thompson D, Ganesan V: Revascularisation surgery for paediatric moyamoya: a review of the literature. Childs New Syst 2005, 21: 358–364.

    Article  Google Scholar 

  86. Bonduel M, Hepner M, Sciuccati G, et al.: Prothrombotic disorders in children with moyamoya syndrome. Stroke 2001, 32: 1786–1792.

    PubMed  CAS  Google Scholar 

  87. Andrew M, Monagle PT, Brooker L: Thromboembolic Complications During Infancy and Childhood, edn 1. Hamilton, Ontario: B.C. Decker Inc.; 2000.

    Google Scholar 

  88. Wang WC, Kovnar EH, Tonkin IL, et al.: High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease. J Pediatr 1991, 118: 377–382.

    Article  PubMed  CAS  Google Scholar 

  89. Sumoza A, de Bisotti R, Sumoza D, Fairbanks V: Hydroxyurea (HU) for prevention of recurrent stroke in sickle cell anemia (SCA). Am J Hematol 2002, 71: 161–165.

    Article  PubMed  CAS  Google Scholar 

  90. National Institutes of Health: Bone Marrow Transplantation in Treating Children with Sickle Cell Disease. Washington, DC: 1999.

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lynch, J.K., Pavlakis, S. & de Veber, G. Treatment and prevention of cerebrovascular disorders in children. Curr Treat Options Neurol 7, 469–480 (2005). https://doi.org/10.1007/s11940-005-0047-6

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11940-005-0047-6

Keywords

Navigation