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SMA Syndrome
Joseph Junewick, MD FACR
over 9 years ago
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Case Detail

Anatomy: Brain-Spine
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 11 years ago
Updated: over 9 years ago
Tags: PEDS
Modality/Study Types: MR CT
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4 month old former 32 week premature twin with extensive portwine stain of entire face and involving the scalp, neck and chest.

Case Images




CT – demonstrates bilateral right greater than left cerebral atrophy and dystrophic junctional calcifications.

MR – demonstrates enlarged subarachnoid spaces with pial angiomatosis. Note the enlarged choroid plexi.


Sturge-Weber is one of the phacomatoses characterized by facial nevus in the distribution of the first division of the trigeminal nerve and leptomeningeal angiomatosis.

Intracranial abnormalities are ipsilateral to the portwine stain. The disease is usually unilateral but 8-15% are bilateral. Leptomeningeal vascular dysplasia results in dilated arteries and venules in the subarachnoid space and non-development of deep cerebral veins. Anomalous venous drainage results in cerebral atrophy, impaired function of the arachnoid granulations and dystrophic calcification. Dystrophic calcification occurs at the gray-white matter junction and in the white matter between layers 2 and 4 but is unusual before 1 year of age.

Choroidal angiomas can also be seen on neuroimaging as thickening of the posterior oculus which is hyperintense on T2 and gadolinium enhanced fat-suppressed T1 images. Angiomas may also be seen in other viscera including the thyroid, lungs, pancreas, spleen and kidneys.


Vilanova JC et al. Hemangiomas from Head to Toe: MR Imaging with Pathological Correlation. Radiographics 2004; 24:267-385.

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