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Infantile hemangiomas: 

    • Present between 2 weeks and 2 months of life, rapid proliferation during first 9 months, gradual involution until 3.5 years
    • Treatment: 
      • Observation: total involution of hemangiomas occur in 50% by age 5, 70% by age 7 and 90% by age 9 
      • Propranolol 
        • Mechanism of action: blocks noradrenaline action on VEGF production 
        • Adverse effects of propranolol: lethargy, hypotension, hypoglycemia, bradycardia
      • Steroids: either oral steroids or intralesional steroids can arrest or slow growth of hemangiomas in the proliferative phase but will not impact involution 
      • Laser: pulse dye Nd:YAG lasers are used for treating ulcerative lesions and for removing residual color from involuted hemangiomas 
      • Surgery: 
        • Usually reserved for older children after involution if there is still excess tissue 
        • Special circumstances: surgery is performed in the proliferative phase if critical structures are threatened including visual obstruction, nasolaryngeal obstruction, or auditory canal obstruction 
    • Special Circumstances: 
      • Parotid tumor infantile hemangiomas frequently are wrapped in facial nerve and difficult to completely resect –> observe 
      • Liver is the most common extracutaneous site
    • Complications: 
      • Bleeding: usually self limited 
      • Ulceration: most common, but only occuring in <5% of patients 

Congenital Hemangiomas: 

    • Definition: rare form of hemangioma that is present at birth
    • Classification: 
      • RICH (rapidly involuting congenital hemangioma) – rapidly involutes after birth 
      • NICH (non-involuting congenital hemangioma) – stable over time, and often has a white grey rim
    • Stains negative for GLUT-1

Kaposiform hemangioendothelioma: 

    • Definition: hemangiomas or diffuse hemangiomatosis that is locally aggressive but not metastasize 
    • Association: Kasabach-merritt syndrome  – profound thrombocytopenia due to platelets being trapped and destroyed within the hemangioma 
    • Treat with chemotherapy and resection (vincrystine)

Syndromes Associated with Hemangiomas: 

  • Mafucci syndrome: endochondromatosis associated with multiple cutaneous hemangiomas 
  • Von hippel-lindau disease: hemangiomas of the retina, hemangioblastoma of the cerebellum, commonly associated with cysts of the pancreas, livers, adrenals and kidneys 
  • PHACE syndrome: large facial hemangiomas associated with Posterior fossa malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta, Eye anomalies 

Other Vascular Malformations: 

  • Port Wine Stain:
    • Definition: capillary malformation most commonly seen on the face in the trigeminal nerve distribution 
    • Treatment:
      • Laser: use pulse dye laser and or Nd:YAG 
      • Pharmacologic: imiquimod 
    • Associated syndromes: 
      • Sturge Webber: large facial port wine stain and leptomeningeal venous malformation and metal retardation 
      • Klippel-Trenaunay Syndrome: extremity port wine stain overlying a deeper venous and lymphatic malformation. Associated with limb length discrepancy
      • Parkes Weber: similar to Klippel Tranaunay Syndrom but with the presence of AV fistulas 
  • Venous Malformation: 
    • Presentation: blue or purple lesion that swell in the dependent position. These are often hormone sensitive and can grow during puberty and pregnancy 
    • Treatment: 
      • Sclerotherapy
      • Laser if superficial or involving the skin 
      • Surgical excision 
  • AVM 
    • Presentation: pulsatile high-flow lesion
    • Diagnosis: MRI and angiography 
    • Progression: can progress to an ulcerated, bleeding lesion that can lead to decompensated heart failure or destructive coagulopathy 
    • Treatment: embolization and surgical resection with WLE because recurrence rates are high 
  • Lymphatic Malformation: 
    • Definition: formation of lymphatic vessels slow flow lesion. Most common on neck and axilla
    • Treatment: 
      • Sclerosis 
      • Surgery 
    • Complications: 
      • Frequent infections requiring abx 

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