Sturge Weber

Reports from the MHC data base

Report from the Observation Chart (PDF)

Synonyms

Encephalotrigeminal angiomatosis.

Codes:

ICD-10: Q85.8B
ORPHA: 3205

Estimated occurrence

2-3:100,000 births

Etiology

Unknown

General symptoms

The syndrome is divided in three subgroups: Sturge Weber type I includes facial and brain angiomas, epilepsy and glaucoma; Sturge Weber type II includes only facial angiomas and glaucoma may occur; Sturge Weber type III includes only brain angiomas. Facial angiomas, a port wine colored birthmark, may be seen on the forehead, upper eyelid and on one side (or sometimes both sides) of the face (facial angiomas). The color is light pink to dark purple and is caused by an overabundance of capillaries around the trigeminal nerve. Brain angiomas are vascular malformations of the brain surface which can cause learning disabilities, behavioral abnormality, hemiparesis/ weakness and headaches/ migraines.

Orofacial/odontological symptoms

Facial angiomas can cause facial asymmetry and in some persons the front teeth of the upper jaw may be offset to the side that does not have the skin lesion. Certain medications for epilepsy can give dry mouth as a side effect, increasing the risk of cavities (tooth decay). Children with Sturge Weber syndrome and neurological impairment may have delayed speech and language development.

Orofacial/odontological treatment

  • Early contact with dental services for intensified prophylactic care and oral hygiene information is essential.
  • Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed.
  • Speech and language difficulties should be treated by a speech therapist.

Sources

  • The rare diseases database of the Swedish National Board of Health and Welfare.
  • The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
  • The Documentation from the Ågrenska national competence centre for rare diseases.
Updated: 2017-09-13 08:33