II. Epidemiology

  1. Incidence: 33 to 40% of newborns (up to 82%)

III. Pathophysiology

  1. Persistent fetal vessels
  2. Dilated dermal capillaries (Telangiectases)

IV. Differential Diagnosis

  1. Port-Wine Stain (Nevus Flammeus)
    1. Contrast with Nevus Simplex which is typically bilateral
    2. Associated syndromes
      1. Klippel-Trenaunay-Weber Syndrome
      2. Sturge-Weber Syndrome
      3. Neonatal Glaucoma
  2. Genetic Syndromes associated Nevus Simplex (small subset, see referral indications under management)
    1. Beckwith Wiedemann Syndrome
    2. Macrocephaly-Capillary Malformation Syndrome
    3. Nova Syndrome
      1. Port-wine nevi-mega cisterna magna Hydrocephalus Syndrome
    4. Odontodysplasia
    5. Roberts-SC Phocomelia Syndrome

V. Signs

  1. Flat vascular patch with indistinct margins
  2. Color may be pale-pink, salmon, bright red or violet
  3. Lesions blanch on compression
  4. Provocative
    1. May become more prominent with crying
  5. Distribution and Course: Often symmetric - involving the bilateral face
    1. Glabellar area (Angel's Kiss)
      1. Resolves spontaneously in 95% of cases
    2. Upper Eyelids
      1. Resolves spontaneously in 100% of cases
    3. Nape of neck ("Stork bite")
      1. Resolves spontaneously in 50% of cases
      2. Persistant lesions will usually cover with hair

VI. Management

  1. No intervention typically needed as these resolve spontaneously in most cases
    1. Most lesions regress by 12 to 18 months (many lesions resolve while still newborns)
  2. Persistent Cases
    1. Flash-Lamp pumped pulse dye laser (FPDL)
  3. Indications for referral (suspected Genetic Syndrome)
    1. Limb Malformation
    2. Macrocephaly
    3. Neurologic symptoms
    4. Seizures

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