Sturge–Weber syndrome

Overview

Board takeaway: Map intraoral vascular involvement before planning surgery or bleeding control. Sturge–Weber is a somatic GNAQ-related vascular disorder of the skin, brain, and eyes.

A forehead/upper-eyelid port-wine birthmark is a recognition clue; not every facial birthmark establishes the syndrome.

How does Sturge–Weber syndrome affect dental management?

• Surgery: Imaging or vascular consult before surgery; local hemostatic plan
• Seizures: Seizure precautions
• Local anesthesia: Careful local anesthetic technique; avoid injecting into vascular lesions
• Anesthesia planning: If anesthesia is needed, coordinate with an experienced team; assess airway vascular involvement and seizure control before choosing the setting and intubation route

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Map gingival, mucosal, and bony involvement before affected-side surgery; use specialist imaging when needed. Establish hemostasis and the setting before treatment, avoiding injection into vascular lesions.

Exam pearl

What is Sturge–Weber syndrome?

A congenital, usually sporadic neurocutaneous disorder involving abnormal blood vessels of the skin and brain, often with a facial port-wine birthmark, leptomeningeal vascular malformation, seizures, and possible glaucoma.

What are the main systemic features of Sturge–Weber syndrome?

• Recognition: Facial port-wine birthmark, often involving the forehead or upper eyelid and usually unilateral; distribution and associated findings determine the need for neurologic and ophthalmologic evaluation
• Associated features: Seizures, hemiparesis, intellectual disability, glaucoma
• Treatment: Treated with anticonvulsants, laser for the stain, glaucoma therapy

What oral findings occur in Sturge–Weber syndrome?

• Ipsilateral vascular lesions of gingiva, lips and buccal mucosa; hemihypertrophy of the jaw
• Gingival enlargement from vascularity and phenytoin
• Accelerated eruption on the affected side; malocclusion

For Sturge–Weber syndrome, how do you plan an affected-side extraction?

• Map the vascular lesion: define whether gingiva, mucosa, or underlying bone is involved with the appropriate oral surgery/vascular team; use imaging when needed to establish extent and blood-flow characteristics.
• Plan hemostasis: prepare local measures and consider a hospital setting.
• Review technique and seizures: avoid injections into vascular lesions; review anticonvulsants and seizure control.

A child has a unilateral port-wine stain over the forehead and upper eyelid, seizures and glaucoma. What is the diagnosis and the dental concern?

• Diagnosis: Sturge–Weber syndrome; somatic GNAQ mutation with characteristic skin, brain, and eye vascular involvement.
• Dental concern: ipsilateral intraoral vascular lesions can hemorrhage during extraction or surgery.

Sources

• Supplemental systemic features: MedlinePlus Genetics: sturge weber syndrome
• Supplemental study guide: Pediatric Dental Study Guide: Sturge-Weber syndrome
• Management Considerations for Pediatric Oral Pathology
• Management of Medical Emergencies