Sturge-Weber syndrome

Case contributed by Julian Yedlin
Diagnosis certain

Presentation

An asymptomatic patient with a history of Sturge-Weber syndrome undergoes a control study.

Patient Data

Age: 20 years
Gender: Female
mri

Presence of focal hypointense images in gradient echo in occipital and right parietal topography is observed.

After intravenous contrast, the presence of leptomeningeal enhancement is described, in relation to leptomeningeal angioma.

Slight decrease in the volume of the right parietal lobe in relation to the contralateral one.

Increased size of the right choroid plexus in the topography of the occipital horn.

The pituitary has a cephalocaudal diameter of approximately 9.5 mm with homogeneous post contrast enhancement. In relation to macroadenoma.

Case Discussion

Sturge-Weber syndrome (SWS) is often associated with neurological complications such as seizures, so the diagnosis of this condition has important implications for patient management.

This neurocutaneous disorder is characterized by angiomatosis of the skin, eye and meninges. The expression of the syndrome is variable but typically manifests as facial port-wine stain (PWS), ocular choroidal hemangiomas and cerebral pial angiomatosis.

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