II. Definitions
- Nevus Flammeus (Port-Wine Stain)
- Persistent vascular Birthmark
III. Epidemiology
- Incidence: 0.3% to 0.5% of newborns (1 in 300)
- Not genetic
IV. Pathophysiology
-
Vascular Malformation of mature ectatic capillaries
- Superficial Capillaries (seen in Salmon Patch)
- Deeper vessels of Dermis and subcutaneous tissue
- Associated with decreased Sympathetic Nerve fibers in the region of the lesions
V. Signs
- Flat, well-demarcated Macules on face or extremities
- Unilateral (rarely cross the midline)
- Present at birth and may deepen in color with time
- Varied coloration and borders
- Blanchable, pale pink to reddish-blue or purple vascular patch
- Irregular borders
- Lesions may progress
- Nodules
- Pyogenic Granuloma
- Soft tissue hypertrophy (e.g. Gingiva)
VI. Differential Diagnosis
VII. Evaluation
- Lesion located near Eyelid requires pediatric ophthalmology evaluation
- Sturge-Weber Syndrome (5-8%)
- Neonatal Glaucoma (10% of lesions that involve the Eyelid)
- Lesion associated with extremity hypertrophy
VIII. Associated Conditions
- Klippel-Trenaunay-Weber Syndrome
-
Sturge-Weber Syndrome
- Overall 1 in 20,000 to 1 in 50,000 children (more common with Eyelid involvement)
- Congenital Neurocutaneous condition
- Associated with CNS and eye Vascular Malformations
- Risk of blindness, focal Epilepsy, Hemiplegia and Learning Disorders
- Neonatal Glaucoma
- When ophthalmic branch of the Trigeminal Nerve is involved
IX. Management
- Treatment is cosmetic only (to lighten lesions)
- Compression garment if on extremities
-
Pulsed-dye laser therapy
- Optimally treated before age 1 year
- Protocol: Total of 5 sessions each spaced 2 weeks apart
- Transient Purpura, edema, crusting or Blistering
- Rare longterm complications (e.g. scarring, pigment changes)
- Tomson (2006) Br J Dermatol 154:676-9 [PubMed]
X. Course
- Persist and may become more prominent over time (contrast with Hemangiomas which fade)