II. Epidemiology
- Incidence: 33 to 40% of newborns (up to 82%)
III. Pathophysiology
- Persistent fetal vessels
- Dilated dermal capillaries (Telangiectases)
IV. Differential Diagnosis
-
Port-Wine Stain (Nevus Flammeus)
- Contrast with Nevus Simplex which is typically bilateral
- Associated syndromes
-
Genetic Syndromes associated Nevus Simplex (small subset, see referral indications under management)
- Beckwith Wiedemann Syndrome
- Macrocephaly-Capillary Malformation Syndrome
- Nova Syndrome
- Port-wine nevi-mega cisterna magna Hydrocephalus Syndrome
- Odontodysplasia
- Roberts-SC Phocomelia Syndrome
V. Signs
- Flat vascular patch with indistinct margins
- Color may be pale-pink, salmon, bright red or violet
- Lesions blanch on compression
- Provocative
- May become more prominent with crying
- Distribution and Course: Often symmetric - involving the bilateral face
VI. Management
- No intervention typically needed as these resolve spontaneously in most cases
- Most lesions regress by 12 to 18 months (many lesions resolve while still newborns)
- Persistent Cases
- Flash-Lamp pumped pulse dye laser (FPDL)
- Indications for referral (suspected Genetic Syndrome)
- Limb Malformation
- Macrocephaly
- Neurologic symptoms
- Seizures