• Hemangiomas: non-malignant neoplasms consisting of vascular endothelial cells and mast cells that enhance neoangiogenesis. Multiple cutaneous hemangiomas may be associated with other sites of hemangiomas, including the liver, lung, and gastrointestinal (GI) tract. Rarely, the meninges, brain, spinal cord, pancreas, spleen, and adrenals may be involved.
• Vascular malformations: capillaries, veins, lymphatics, or arteries that underwent errors of morphogenesis (i.e., hamartomas); includes port-wine stains (nevus flammeus) and arteriovenous malformations. Port-wine stains can signal underlying developmental defects especially of central nervous system and spine.
• Salmon patches (nevus simplex): distended dermal capillaries (nevus simplex, telangiectatic nevus).

• Hemangiomas: appear in first few weeks of life, grow rapidly in first 6 to 12 months of life, followed by static period, and then involution.
• Vascular malformations: grow proportionally with the affected individual. Congenital by definition, may not become apparent until adolescence or adulthood, port-wine stain apparent at birth.
• Salmon patches: Grow proportionally with affected individual.

• Hemangiomas
  • Usually no genetic association when isolated finding
  • Seen with Klippel-Trénaunay syndrome, Parks-Weber syndrome, Maffucci syndrome, blue rubber bleb nevus syndrome (dominant inheritance), diffuse neonatal hemangiomatosis, Cobb syndrome
• Vascular malformations
  • Seen with Sturge-Weber syndrome, Cobb syndrome, Wyburn-Mason (or Bonnet-Dechaume-Blanc) syndrome, Klippel-Trénaunay syndrome, Parks-Weber syndrome
• Salmon patches
  • No genetic association

• Hemangiomas
  • Female:male ratio 3:1
  • Increased incidence with very low birthweight (<1000 g) infants
  • Present in up to 12% of infants
  • 80% are single lesions
  • 60% are found on head and neck
  • 25% are found on trunk
• Vascular malformations
  • Port-wine stains female:male ratio 1:1
• Salmon patches
  • Found in 30% to 40% of all newborns

• Hemangiomas
  • Local ulceration, bleeding, superinfection
  • Obstruction of important structures including eye, airway, GI tract
  • High output cardiac failure in presence of large number of lesions
  • Iron-deficiency anemia secondary to hemorrhage from GI lesions
  • Kasabach-Merritt syndrome: platelet trapping, coagulopathy, thrombocytopenia; rarely cause skeletal overgrowth secondary to increased blood flow
• Vascular malformations
  • Can lead to “steal” causing ischemic necrosis, pain, and increased cardiac output
  • Frequently associated with skeletal abnormalities including hypertrophy (low flow) or destruction (high flow)
  • Localized or disseminated intravascular coagulopathy possible, but uncommon

• Hemangiomas: excellent except when associated with other syndromes.
• Vascular malformations: varied depending on type of malformation and associated syndromes.
• Salmon patches: excellent.

• Bacillary angiomatosis

• Glomus tumor
• Angioendothelioma
• Sarcoma

• Verrucous hemangioma

• Pyogenic granuloma
• Angiokeratomas
• Acquired tufted angioma

Question: Hemangiomas
Significance: Present in first few weeks of life, but not at birth, rapid growth noted in first year of life.

Question: Vascular malformations
Significance: Most present at birth, sometimes not noted until adolescence or adulthood.

Question: Salmon patches
Significance: Noted at birth.

Finding: Young lesion in superficial dermis
Significance: Raised, bright red-pink color, sharply demarcated border, stippled surface—hence term “strawberry hemangioma”

Finding: Young lesion in lower dermis or subcutaneous tissue
Significance: Bluish in color, raised with smooth overlying skin

Finding: Superficial and deep components
Significance: Can be combined in a single lesion

Finding: Involuting lesion
Significance: Develops gray or white areas, usually centrally located, which spread toward periphery

Finding: Overlying spine
Significance: Raises concern of underlying pathology

Finding: Port-wine stains
Significance: Apparent at birth, pink-to-red sharply demarcated macule, possibly slightly raised, darkens with age, may develop papules over time

Finding: Other vascular malformations
Significance: May not be noticed until adolescence or adulthood and are usually soft, easily compressed, and emptied of blood.

Finding: Salmon patches
Significance: Often have more than one lesion; 80% on nape of neck, 45% on eyelids, 33% on glabella.

• Occasionally necessary to differentiate hemangiomas from vascular malformations
• Head MRI: performed to rule out anomalies of the choroid plexus and leptomeninges (Sturge-Weber); must be performed when port-wine stains include first distribution of the trigeminal nerve. Lesions in second and third distribution of trigeminal nerve do not have this risk.

• Hemangiomas
  • For bleeding: direct pressure
  • For ulceration: observation or wet compresses and topical antibacterials
  • For infection: intravenous antibiotics for cellulitis, topical antibiotics with dressing changes for mild involvement
  • Treatment is necessary for lesions that interfere with critical structures (e.g., eye, airway, GI tract) or for serious symptoms, such as consumptive coagulopathy. No therapy indicated in 95%.
  • Intralesional or systemic steroids
  • Interferon-alpha-2a for steroid non-responders
  • Liquid nitrogen, laser therapy occasionally beneficial
  • Surgical resection utilized infrequently
• Vascular malformations
  • Port-wine stain: cosmetic camouflage or laser therapy. Laser therapy is being continuously improved in terms of quality of outcome and decreased complications as different types of lasers are developed and tested. Multiple laser treatment sessions at 2- to 3-month intervals are usually necessary.

• Hemangiomas
  • 50% will spontaneously resolve by 5 years.
  • 70% will spontaneously resolve by 7 years.
  • 90% will spontaneously resolve by 9 to 12 years.
• Vascular malformations
  • No spontaneous resolution of lesions.
• Salmon patches
  • 50% on nape of neck spontaneously resolve.
  • 95% of others spontaneously resolve.
  • Tendency toward erythema in affected area may persist after lesions resolve.

• For hemangiomas, treatment significantly increases the risk of poor cosmetic outcome.
• For vascular malformations, treatment of port-wine stains may achieve color lightening rather than complete resolution of the lesion.

Q: What will happen to my child’s hemangioma?
A: Expect it to enlarge, get a bluish hue, and then get smaller. In the first year of life, it may enlarge at a rate more rapid than the rest of the child’s growth. After that time it will involute, and should resolve by 9 to 12 years of age; most resolve earlier.

Q: What are the problems of the involuting hemangioma?
A: Infection and ulceration.

Q: What happens to the “stork bite” on the back of the neck?
A: It fades but still may be seen when vessels dilate.