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[Sturge-Weber syndrome: experience with 14 cases]
J Miranda Mallea1, I Güemes Heras, P Barbero Aguirre
1Servicio de Neuropediatría, Hospital Infantil La Fe, Valencia.
Anales Espanoles De Pediatria
|February 1, 1997
Summary
Early evaluation and treatment are crucial for Sturge-Weber syndrome (SWS) patients, especially those with facial nevus flammeus. Prompt diagnosis and intervention improve outcomes for SWS, a rare neurological disorder.
Area of Science:
- Neurology
- Pediatrics
- Ophthalmology
Context:
- Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by facial port-wine stains and neurological abnormalities.
- Diagnosis typically involves clinical presentation, neuroimaging, and ophthalmological examination.
Purpose:
- To review clinical characteristics, disease progression, and treatment responses in SWS patients over 25 years.
- To evaluate the efficacy of various therapeutic interventions for SWS manifestations.
Summary:
- This retrospective study analyzed 14 SWS patients, with 11 diagnosed before 18 months. Seizures (13/14) and hemiparesis (9/14) were common, often contralateral to the facial nevus flammeus.
- Good therapeutic response was observed in 10/13 patients treated for seizures. MRI proved superior to CT in detecting parenchymal atrophy and gray/white matter abnormalities.
- Glaucoma occurred in 4 patients, with 3 showing good outcomes post-trabeculotomy.
Impact:
- Highlights the importance of early neuroimaging (MRI) and ophthalmological assessment in newborns with trigeminal nerve- V1 distribution facial nevus flammeus.
- Emphasizes the need for prompt diagnosis and management of SWS to mitigate neurological deficits and improve patient outcomes.
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