Hemangiomas and Other Vascular Lesions

Hemangiomas and Other Vascular Lesions is a topic covered in the Select 5-Minute Pediatrics Topics.

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  • Vascular tumors: neoplasms of the vasculature
    • Infantile hemangioma (IH)
    • Congenital hemangiomas: noninvoluting congenital hemangioma (NICH) and rapidly involuting congenital hemangioma (RICH)
    • Tufted angioma (TA)
    • Kaposiform hemangioendothelioma (KHE)
    • Pyogenic granuloma
    • Hemangiopericytoma
  • Vascular malformations (VaM): anomalous blood vessels without endothelial proliferation
    • Capillary malformations (CM) (e.g., salmon patch, port-wine stain, nevus flammeus)
    • Venous malformations (VM)
    • Arterial malformations: arteriovenous malformations (AVM) or arteriovenous fistula (AVF)
  • Lymphatic malformations (LM) (macrocystic and microcystic)
  • Combined malformations (e.g., capillary-venous-lymphatic)
  • Other types of VaM may occur in any part of the body and may be associated with soft tissue overgrowth of the involved part.



Infantile hemangiomas

  • 4–5% of infants
  • ∼10% of Caucasian infants by age 12 months
  • Increased incidence in low-birth-weight and premature infants
  • Other demographic risk factors include white non-Hispanic race, female sex, multiple gestation pregnancy, advanced maternal age, chorionic villus sampling during pregnancy, and positive family history.

Commonly Associated Conditions

  • Infantile hemangiomas
    • PHACES
      • Segmental hemangioma (usually facial) associated with other developmental anomalies (Posterior fossa malformations; hemangiomas; arterial anomalies; cardiac anomalies, including aortic coarctation; eye abnormalities; sternal defects/supraumbilical raphe)
      • Cerebral malformations occur in >50% and cerebrovascular anomalies in 33%.
    • Lumbosacral
      • An underlying spinal dysraphism may be present.
      • PHACE-like syndromes of the lower body with associated GI and GU anomalies have been described.
    • Segmental
      • Commonly located on the face and involving a developmental unit (segment) and frequently associated with complications, even without meeting full PHACES criteria
      • Segmental IHs are much more likely to have complications and to receive treatment than focal IHs.
  • Kaposiform hemangioendothelioma
    • Kasabach-Merritt phenomenon (consumptive coagulopathy, severe thrombocytopenia)
  • VaM
    • CM may be present as part of syndromes (e.g., Sturge-Weber, RASA-1 mutations associated with fast-flow VaM, von Hippel-Lindau, Rubinstein-Taybi, Beckwith-Wiedemann, Cobb syndrome).
    • VM can be inherited autosomal dominantly due to TIE2/TEK gene mutations.
    • CM-AVM: due to RASA-1 gene mutation and associated with fast-flow VaM
    • Generalized LM and lymphedema have been associated with VEGFR3 mutations.

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