Definition: Sturge-Weber Syndrome (encephelotrigeminal angiomatosis) is a congenital, non-familial hamartomatous vescular proliferation mainly involving areas along the trigeminal nerve distribution.
Etiology: Not clear, but is thought to be caused by the presence of a vascular plexus around the portion of the neural tube destined to become the facial skin.
Frequency: Rare.
Clinical Features: Hemangiomas of the skin, face, and oral mucosa, calcifications of the brain, ocular disorders such as glaucoma, epilepsy and mild mental retardation. Facial hemangiomas are the most constant and characteristic finding of this syndrome appear as a bright red or purple and are usually unilateral.
Etiology: Not clear, but is thought to be caused by the presence of a vascular plexus around the portion of the neural tube destined to become the facial skin.
Frequency: Rare.
Clinical Features: Hemangiomas of the skin, face, and oral mucosa, calcifications of the brain, ocular disorders such as glaucoma, epilepsy and mild mental retardation. Facial hemangiomas are the most constant and characteristic finding of this syndrome appear as a bright red or purple and are usually unilateral.
Oral Manifestations: Oral hemangiomas also tend to be unilateral, may involve the maxillary gingival, buccal mucosa, tongue and lips, are usually flat, but may have a raised irregular surface that causes tissue enlargement. Delayed or early and ectopic dental eruptions are also common.
Treatment: Depends on the location and severity of the lesions, laser therapy may lighten or remove stains. Anticonvulsants may be indicated.
Treatment: Depends on the location and severity of the lesions, laser therapy may lighten or remove stains. Anticonvulsants may be indicated.
Dental Considerations: Symptomatic.
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