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Published on: September 19, 2019
[Sturge-Weber syndrome. Early manifestation and visualization of disease course]
E Stranzinger1, T A G M Huisman
1Abteilung Bilddiagnostik, Universitäts-Kinderklinik, Steinwiesstr. 75, CH-8032 , Zürich, Schweiz. enno.stranzinger@kispi.unizh.de
Insights
Ultrasound can detect early signs of Sturge-Weber syndrome in newborns. Magnetic resonance imaging (MRI) is the preferred method for diagnosing and monitoring this condition in children.
Area of Science:
- Pediatric neurology
- Neuroradiology
- Medical imaging
Context:
- Sturge-Weber syndrome is a rare congenital disorder.
- Early diagnosis and monitoring are crucial for managing neurological complications.
- Imaging plays a vital role in assessing the extent of the disease.
Purpose:
- To evaluate the effectiveness of ultrasound, CT, and MRI in diagnosing Sturge-Weber syndrome in children.
- To identify the earliest imaging indicators of the syndrome.
- To determine the optimal imaging modality for diagnosis and follow-up.
Summary:
- Ultrasound revealed periventricular white matter echogenicity in neonates, suggesting an early sign of Sturge-Weber syndrome.
- MRI demonstrated vascular malformations, impaired cerebral venous drainage, and hemispheric atrophy.
- CT scans were also reviewed as part of the comparative analysis.
Impact:
- One-sided periventricular hyperechogenicity on ultrasound may indicate Sturge-Weber syndrome in infants with nevus flammeus.
- MRI is the gold standard for diagnosing Sturge-Weber syndrome and for monitoring disease progression or changes in neurological status.
Background:
The purpose of this study was to evaluate ultrasound, MRI, and CT investigations in children with Sturge-Weber syndrome.
Patients And Methods:
From 1996 to 2005, a total of 15 investigations of the brain performed in 6 children with Sturge-Weber syndrome were reviewed. We evaluated four ultrasound, five CT, and six MRI exams.
Results:
With ultrasound an increase of the echogenicity of the periventricular white matter was depicted in the first days of life. MRI is the best modality to demonstrate the vascular malformation, the impaired cerebral venous drainage, and the atrophy of one hemisphere.
Conclusions:
One-sided periventricular hyperechogenicity on ultrasound can be an early sign of Sturge-Weber syndrome in children with a nevus flammeus. MRI is the method of choice to diagnose Sturge-Weber syndrome and to follow up these children if the neurological status of the patients changes.
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