Presentation
Right-sided face port wine stain.
Patient Data
Age: 2 months
Gender: Male
From the case:
Sturge-Weber syndrome
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MRI mild asymmetry in myelination at the right frontoparietal region.
Contrast study shows prominent leptomeningeal enhancement reflecting pial angiomatosis.
Case Discussion
Sturge-Weber syndrome, or encephalotrigeminal angiomatosis, has three major features: port-wine stain, pial angiomatosis, and glaucoma.
Contrast-enhanced MRI is performed in asymptomatic infants with a facial port-wine stain for early detection of Sturge-Weber syndrome.
Later in life, brain atrophic changes and ipsilateral choroid plexus hypertrophy will be noticed.
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