Sturge-Weber Syndrome

Incidence: 1 in 5000.

Prevalence: No racial or sex prediction; sporadically occurring neurocutaneous syndrome.

Increased risk of seizures, neurologic deficits, bleeding due to presence of angiomas involving the oral cavity, vascular abnormality, and congenital cardiac malformations.

Seizures, mental retardation, neurologic deficits, headache

Congenital glaucoma, retinal detachment

Difficult airway

Intracerebral angiomas

Described by Sturge (1879) and Weber (1929).

Also known as encephalotrigeminal angiomatosis.

Involves a triad of (1) vascular malformation (port wine stain); (2) leptomeningeal angioma; and (3) vascular malformation of the eye.

Facial, extrafacial, and bilateral port wine stain, along with hypertrophy of the facial soft tissue and facial bone:

• Obstructive sleep apnea.

• Difficult mask ventilation and laryngoscopy.

Seizure:

• The earlier the onset, the poorer the prognosis.

• May need multiple antiepileptic drugs.

• Treatment of dehydration/fever/infection.

Mental retardation leads to anxiety, agitation.

Hemiparesis, hemianopsia, hemiplegia.

Status-like episodes.

Unknown. Suggested etiology includes

• Failure of primitive cephalic venous plexus to regress during first trimester of pregnancy

• Failure of superficial cortical veins to develop

• Thrombosis of veins leading to vascular steal phenomena

• Deficiency of sympathetic insertion of vessel