Authors: John Morgan, Bonnie F. Zimble
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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
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Study
Questions
- What are the clinical manifestations of
Sturge-Weber Syndrome?
- Describe
it oral manifestations.
- Summarize the four features of dental management.
- 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
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