Sturge-Weber Syndrome, Port Wine Stain, Kasabach-Merritt, Klippel-Trenaunay-Weber, type-1 (NF-1), tuberous sclerosis)

Sturge-Weber syndrome (SWS)

  1. Sporadic occurrence
  2. Facial port-wine stain in CN 5 distribution
  3. Ipsilateral leptomeningeal angiomas w/ intracranial calcifications
  4. High incidence of seizures, MR and behavior problems
  5. Ocular problems (buphthalmos, coloboma, Glaucoma)

Although infants who have Kasabach-Merritt or Klippel-Trenaunay-Weber syndromes have cutaneous vascular anomalies, clinical features of these disorders permit their differentiation from SWS.

Infants who have neurofibromatosis type-1 (NF-1) or tuberous sclerosis do not exhibit characteristic cutaneous vascular anomalies.

Enjolras O, Mulliken JB. Vascular malformations. In: Harper J, Oranje
A, Prose N, eds. Textbook of Pediatric Dermatology. Malden, Mass:
Blackwell Science, Ltd; 2000:975-1016
Orlow SJ. When to suspect a neurocutaneous disorder. Contemp Pediatr.
Tallman B, Tan OT, Morelli JG, et al. Location of port-wine stains and
the likelihood of ophthalmic and/or central nervous system
complications. Pediatrics. 1991;87:323-327