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MR-imaging findings in children with Sturge-Weber syndrome
J Sperner1, I Schmauser, R Bittner
1Children's Hospital, Kaiserin Auguste Victoria Haus, Berlin.
Insights
Magnetic resonance imaging (MRI) with Gadolinium-DTPA (Gd-DTPA) enhancement is crucial for visualizing leptomeningeal angiomatosis in children with Sturge-Weber syndrome (SWS). This method effectively detects intracranial involvement before CT can identify calcifications, aiding early diagnosis and management.
Area of Science:
- Pediatric Neurology
- Neuroradiology
- Medical Imaging
Background:
- Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by facial port-wine stains, neurological abnormalities, and vascular malformations.
- Leptomeningeal angiomatosis, a key feature of SWS, involves abnormal blood vessels in the brain's meninges, potentially leading to seizures and developmental delays.
Abstract:
Intracranial extent and distribution of leptomeningeal angiomatosis, visualized by magnetic resonance imaging (MRI) with Gadolinium-DTPA (Gd-DTPA) enhancement, is demonstrated in four children with Sturge-Weber syndrome (SWS). Aged 7, 9, 11 and 19 months, they presented with cutaneous, neurologic and ocular symptoms at the time of MRI examination. Angiomatous alteration of the skull, atypically located and congested intracerebral and basal veins as well as intracerebral changes secondary to the leptomeningeal angiomatosis are demonstrated with T2 weighted images. Gd-DTPA enhanced T1 weighted images exhibit clearly the regional distribution of angiomatosis in the skull, meninges and within the brain. Before calcifications in children with SWS are detectable by CT, MRI is the method of choice to detect intracranial involvement. Enhancement with Gd-DTPA improves the diagnostic value of MRI, before neurological symptoms appear. Follow-up studies with Gd-DTPA enhanced MRI can be applied to recognize thrombotic changes of leptomeningeal angiomatosis as well as subsequent intracerebral impairment.