What Are the Secondary Manifestations?

Psychological - Children and adolescents with Sturge-Weber syndrome are at risk for intellectual, academic and behavioral problems. There are certainly children with this disorder who function well in both academic and social settings but the risk of problems is much higher than it is for children in the general population There is a high incidence of Attention Deficit/Hyperactivity Disorder/PDD in young people with SWS (20% vs 5-6% in the general population). There have been reports of an elevated frequency of Autism in individuals with SWS although older children and adolescents are at risk for depression.

Oral - Increased Vascularity of the hard & soft tissues of the jaw on the involved side of the face may occur. Swelling of the oral soft tissues is a fairly common manifestation of SWS. Other oral findings associated with SWS include enlargement of one side of the jawbone containing the teeth (on the same side as the facial lesion) and a high arched palate, although one sided enlargement or diminished jaw size opposite to the facial lesion have been reported. The increased vascularity of the bones of the jaw may also lead to premature eruption of permanent teeth, unusual eruption time and location of teeth, and periodontal problems such as pockets and loss of attachment of teeth to jaw, resulting in mobility and premature loss of teeth.

Endocrine – It has been found there is a growth hormone deficiency prevalence of 61% in SWS patients, which is 21 fold higher than the general population prevalence of .03%. In addition, other endocrine abnormalities have been identified, however further studies need to be undertaken.

Headache - About half of the patients complain of headaches. Migraine represents the most frequent type of headache. Headaches commonly occur after epileptic seizures. In a few patients headaches may be due to glaucoma.