Presentation
Patient with port-wine stain of the left side of the face (involving the ophthalmic division (V1) of the trigeminal nerve) and thorax with left hemihypertrophy. Seizures with onset during the neonatal period. Presentation for follow-up.
Patient Data
Pronounced leptomeningeal as well as pachymeningeal contrast enhancement, predominantly of the left cerebral hemisphere with also minor leptomeningeal enhancement of the parafalxial right cerebral hemisphere. Severe hypotrophy of the left cerebral hemisphere as well as the right cerebellar hemisphere (possibly due to crossed cerebellar diaschisis). Finely dispersed susceptibility artifacts of the left subcortical parenchyma, suspicious for calcification. Enlargement of the left choroid plexus. Enhancement of the left ocular fundus and extraocular musculature.
In conjunction with the clinical presentation, the findings are consistent with a Sturge-Weber syndrome with leptomeningeal angiomatosis and cerebral calcifications, as well as associated left-sided choroidal angioma.
Case Discussion
Sturge-Weber syndrome remains a clinical diagnosis. However, imaging can help confirm the diagnosis, assess the extent of the disease, and look for clinically relevant abnormalities. In this case, choroidal hemangioma is an important diagnosis (with the risk of retinal or choroidal detachment due to persistent exudation) that can be difficult to make clinically.
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