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The Cutaneous Angioma of Sturge-Weber Syndrome

A 16-month-old male presented with focal motor seizures with secondary generalization. On physical exam, a bilateral facial cutaneous angioma, sharply demarcated by the distribution of the ophthalmic and maxillary branches of the trigeminal nerve, was noted (Figure 1). Magnetic resonance imaging (MRI) demonstrated calcification and atrophy of the right frontal lobe as well as an associated pial angioma, consistent with the clinical suspicion of Surge-Weber syndrome (SWS) (Figure 2). However, no abnormality was seen in the right hemisphere, or in the parietal or occipital lobes. Further evaluation also revealed unilateral glaucoma on the right, but with bilateral diffuse choroidal hemangiomas. Surge-Weber syndrome is a non-hereditary neurocutaneous disorder characterized by leptomeningeal angiomatosis with an associated facial cutaneous angioma, also called a port-wine nevus. The abnormality is thought to be due to a malformation of the embryonic vascular plexus within the cephalic mesenchyme at five to eight weeks gestation, interfering with the development of vascular drainage.1 Due to poor drainage of the eye, face, leptomeninges and brain, angiomatosis develops as an alternate pathway for venous drainage,1 leading to multiple neurological and ophthalmologic complications, including seizures, headaches, stroke like episodes and glaucoma.