Hemangiomas:
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