Imaging Sturge-Weber Syndrome with the Feed-and-Swaddle FAST Protocol

Sturge-Weber syndrome is a rare, mosaic neurocutaneous disorder characterized by aberrant microvascular development of the skin, brain, and eyes. Patients present at birth with a facial capillary malformation, commonly called a port-wine birthmark, typically involving the forehead distribution. Intracranial involvement primarily consists of leptomeningeal angiomatosis and is associated with early onset seizures, stroke-like episodes, progressive hemispheric injury, and neurodevelopmental delay. Early onset seizures occur in approximately 75-90% of children and correlate with poor long-term neurologic outcome. Brain MRI is the reference standard for evaluating infants with suspected Sturge-Weber syndrome. Current consensus guidelines recommend screening MRI at 1 year of age in high-risk infants; however, this paradigm creates a critical diagnostic gap. Our institutional experience indicates that performing early MRI screening during the pre-symptomatic window facilitates early referral to neurology, parental education with faster recognition and treatment of seizures, initiation of low-dose aspirin therapy, and early identification of severe hemispheric disease for surgical planning. However, conventional MRI protocols can be lengthy and poorly tolerated by children, necessitating sedation to avoid motion artifacts and obtain qualitative images. To address these limitations, a rapid, disease-targeted MRI approach, called Focused Abbreviated Survey Techniques (FAST), has been developed at our institution and permits the delivery of clinically essential information with markedly reduced acquisition times and improved patient tolerance. In infants with suspected Sturge-Weber syndrome, FAST MRI can detect intracranial involvement without sedation using the feed-and-swaddle technique. In this clinical report, we summarize our experience with FAST MRI in infants with suspected Sturge-Weber syndrome.