Pathologic arterial or venous intravascular occlusion secondary to abnormal thrombus formation. The following are common thrombotic events:

  • Deep venous thrombosis (DVT): involves large systemic veins outside the central nervous system (CNS)
  • Cerebral sinovenous thrombosis (CSVT): involves intracranial venous sinuses
  • Ischemic stroke: CNS arterial occlusion with infarction of brain tissue
  • Intracardiac thrombosis: mural, valvular, or foreign body associated
  • Femoral artery thrombosis: can be associated with vessel catheterization
  • Renal vein thrombosis: commonly in the neonatal period; may be unilateral or bilateral
  • Myocardial infarction: Kawasaki disease, antiphospholipid antibody syndrome, or with severe familial hypercholesterolemia
  • Budd-Chiari syndrome: thrombosis of the hepatic vein
  • Portal vein thrombosis: most commonly seen in neonates with umbilical catheters


  • Incidence of venous thrombosis in children is estimated at 4.9 per 100,000 per year.
  • Age distribution is bimodal; peak rates are found in the neonatal and adolescent age groups.
  • Idiopathic thrombosis is rare in children.
  • >90% of pediatric venous thrombosis is associated with additional risk factors.
  • Central venous lines are the most common risk factor for venous thrombosis in children.

Risk Factors

  • Neonatal
    • Prematurity
    • Maternal diabetes
    • Umbilical catheters or other central lines
    • Sepsis
    • Polycythemia
    • Perinatal asphyxia
  • Malignancy/bone marrow disorders
    • Leukemia (hyperleukocytosis, acute promyelocytic leukemia)
    • Myeloproliferative disorders
    • Paroxysmal nocturnal hemoglobinuria
  • Medications
    • L-Asparaginase
    • Oral contraceptives (with estrogen)
    • Heparin-induced thrombocytopenia
    • Steroids
  • Anatomic
    • Indwelling catheters
    • Congenital heart disease
    • Prosthetic heart valves
    • Intracardiac baffles
    • Tumor compression
    • Atresia of the inferior vena cava
    • Thoracic outlet obstruction (Paget-Schroetter syndrome)
    • May-Thurner syndrome (compression of the left iliac vein by the right iliac artery)
  • Miscellaneous
    • Infection
    • Trauma
    • Surgery
    • Obesity
    • Prolonged immobilization or paralysis
    • Dehydration
    • Antiphospholipid syndrome
    • Inherited prothrombotic state
  • Risk factors/conditions specific for arterial disease
    • Kawasaki disease
    • Takayasu arteritis
    • Hyperlipidemia
    • Antiphospholipid syndrome

Commonly Associated Conditions

  • Nephrotic syndrome
  • Inflammatory disorders
  • Liver disease
  • Sickle cell disease
  • Diabetes mellitus
  • Congenital heart disease (hypoplastic left heart syndrome)

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