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Sturge-Weber syndrome: deep venous occlusion and the radiologic spectrum
Shira E Slasky1, Shlomo Shinnar, Jacqueline A Bello
1Department of Radiology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, NY 10467, USA. shiraeva@yahoo.com
Abstract:
Sturge-Weber syndrome is a neurocutaneous syndrome with a facial port-wine nevus and neurologic features, typically including seizures and hemiparesis. Glaucoma may also occur. MRI features include leptomeningeal angiomatosis, cortical and pial calcifications, and angiomatous change of the choroid plexus. We reviewed a subset of patients with Sturge-Weber syndrome with the rare finding of deep venous occlusion, and present such a case, unusual by comparison to previously reported cases of Sturge-Weber syndrome with deep venous occlusion. Six previously reported cases were reviewed. All cases presented with seizures; five of six had evidence of leptomeningeal angiomatosis; half had cerebral hemiatrophy. This report presents a unique case lacking clinical seizures, but with a port-wine stain and congenital glaucoma. This patient lacked the radiologic findings of leptomeningeal angiomatosis and hemicerebral atrophy, but demonstrated deep venous occlusion with frontal venous collaterals. There is a wide spectrum of findings in Sturge-Weber syndrome. The lack of seizures and angiomatosis in this case are likely "true-true" and related. The case illustrates the unusual finding of deep venous occlusion in Sturge-Weber syndrome occurring without leptomeningeal angiomatosis. Additionally, it demonstrates that although the initial evaluation is normal, patients may later manifest clinical characteristics of Sturge-Weber syndrome.
Insights
Sturge-Weber syndrome, a rare neurocutaneous disorder, can present atypically. This case highlights deep venous occlusion without typical seizures or angiomatosis, expanding the known spectrum of findings.
Area of Science:
- Neurology
- Genetics
- Dermatology
Background:
- Sturge-Weber syndrome (SWS) is a congenital neurocutaneous disorder characterized by facial port-wine stains, neurological abnormalities (seizures, hemiparesis), and potential glaucoma.
- Typical neuroimaging in SWS reveals leptomeningeal angiomatosis, cortical/pial calcifications, and choroid plexus abnormalities.
- Deep venous occlusion is a rare complication within the SWS spectrum.
Observation:
- A unique case of SWS is presented, featuring a port-wine stain and congenital glaucoma but lacking clinical seizures.
- This patient did not exhibit typical radiologic findings of leptomeningeal angiomatosis or hemicerebral atrophy.
- The case demonstrated deep venous occlusion with frontal venous collaterals, an unusual presentation.
Findings:
- Review of six prior cases of SWS with deep venous occlusion showed seizures (all cases) and leptomeningeal angiomatosis (5/6).
- The presented case is unusual for its absence of seizures and leptomeningeal angiomatosis, despite deep venous occlusion.
- This suggests a broader spectrum of SWS manifestations than previously recognized.
Implications:
- The findings expand the understanding of Sturge-Weber syndrome's diverse clinical and radiological presentations.
- Deep venous occlusion can occur in SWS even without seizures or leptomeningeal angiomatosis.
- Patients with SWS may develop clinical features later, even with initially normal evaluations, necessitating long-term monitoring.
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