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Free Content Encephalotrigeminal Angiomatosis: A Review with Emphasis on Head and Neck Manifestations

Encephalotrigeminal angiomatosis, also known as Sturge-Weber syndrome or Sturge-Weber-Dimitri disease, is a sporadic congenital neurocutaneous syndrome characterized by angiomas of the face, eyes, and meninges. The typical clinical findings include seizures, a port-wine facial nevus, hemiparesis, and developmental delay. The classic intracranial imaging features of Sturge-Weber syndrome include cerebral hemispheric atrophy, hypervascularity and/or calcification of the cortex, and ipsilateral choroid plexus enlargement. Extracranial findings primarily include the vascular abnormalities of the ocular and facial soft tissues as well as hypertrophy of the calvarial and maxillofacial osseous structures. Additionally, we report involvement of the extraocular muscles, salivary glands, and lacrimal glands, findings that have not been well described in the literature. While a developmental venous pathology underlying Sturge-Weber syndrome has been proposed resulting in many of the findings, the mechanism behind the facial soft-tissue abnormalities and the clinical sequelae associated with these glandular abnormalities is still uncertain.

Learning Objective: To recognize typical clinical and various imaging manifestations of Sturge-Weber syndrome, including head and neck and intracranial imaging findings

Keywords: PWS = port-wine stain; SWS = Sturge-Weber syndrome

Document Type: Research Article

Publication date: May 1, 2021

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  • Neurographics is the peer-reviewed, quarterly educational journal of the American Society of Neuroradiology. The journal includes review articles as well as high-yield case reports that have been solicited from society meetings, including the annual meeting of the ASNR as well as the American Society of Spine Radiology, the American Society of Pediatric Neuroradiology, the American Society of Functional Neuroradiology, and the American Society of Head and Neck Radiology meetings. Unsolicited educational review articles and case reports are also accepted for review at the discretion of the Editor-in-Chief. Submissions focusing on a pictorial approach to educational objectives are highly encouraged. The journal is open access and available online. CME credit is offered for reading review articles and completing activity evaluations through the ASNR Education Connection website:
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