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Authors: John Morgan, Bonnie F. Zimble

Sturge-Weber Syndromes
:
Clinical and Oral Manifestations, Dental Management and

 Treatment


Special Care in Dentistry
John Morgan, DDS
Tufts University School of Dental Medicine, 2008


Study Questions 

  1. What are the clinical manifestations of Sturge-Weber Syndrome?
  2. Describe it oral manifestations.
  3. Summarize the four features of dental management.
  4. Summarize treatment options for the key manifestations of Sturge-Weber Syndrome.

What are the clinical manifestations of Sturge-Weber Syndrome? 

  • Sturge-Weber Syndrome affects both sexes equally
  • At present, no preventive measures seem to be available, and the disease can’t be determined before birth
  • Naevus flammeus is usually present at birth
  • Seizures begin between 3-9 months of age

What are its oral manifestations? 

  • Oral manifestations occur in about 40% of patients
  • Bluish-red lesion that blanches on pressure
  • Mostly on the buccal mucosa and lips, less on maxillary gingival and palate
  • Floor of the mouth, tongue and mandibular gingiva are rarely involved
  • The lesions resemble a vascular hyperplasia or large tumor-like mass
  • Early eruption of teeth reported from increased vascularity
  • Dilantin hyperplasia is common
  • Oral hygiene problems in MR patients

Dental Management 

  • Major problem is hemorrhage from the angiomas
  • Hospitalization with complete workup is recommended
  • For GA, oral intubation may be safer
  • Special hygiene aids for MR patients

Treatment 

According to the National Institute of Neurological Disorders and Stroke (NINDS):

“Treatment for Sturge-Weber syndrome is symptomatic.  Laser treatment may be used to lighten or remove the birthmark.   Anticonvulsant medications may be used to control seizures.  Surgery may be performed on more serious cases of glaucoma.  Physical therapy should be considered for infants and children with muscle weakness.  Educational therapy is often prescribed for those with mental retardation or developmental delays.  Doctors recommend yearly monitoring for glaucoma.”

In summary, treatment may have the following characteristics ... 

  • Conservative
  • Surgery for glaucoma
  • Dilantin to control seizures
  • Laser therapy for naevus flammeus

 Click here to access the NINDS web site on Sturge-Weber Syndrome