Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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April 18, 2019
Multiple cavernomas of the corpus callosum
- Background
- Cavernous malformations are composed of dilated, thin walled capillaries in apposition, without intervening normal neural tissue, and contain internal hemorrhage of varying ages.
- Approximately 20% of cases are multiple, including familial.
- Often associated with developmental venous anomalies.
- Important, underlying cause of spontaneous intracranial hemorrhage.
- Clinical Presentation
- Presence and type of symptoms dictated by location and growth of the lesion.
- May present with seizure, hemorrhage, headache, focal neurological defect, or be asymptomatic (up to 20%).
- Deep location risk factor for “clinical aggressiveness,” but not necessarily hemorrhage .
- Key Diagnostic Features
- CT: hyperdense lesion, +/- calcifications (~50%)
- MRI: characteristic “popcorn” appearance, with mixed internal T1 hypersignal, reticulated T2 hypersignal, and dark T2 hemosiderin rim; prominent blooming on GRE/SWI; +/- associated DVA (GRE/SWI or contrast-enhanced imaging); surrounding edema of FLAIR/T2 can indicate recent hemorrhage/growth
- Differential Diagnosis (Corpus callosum/calloseptal interface lesions:)
- Hemorrhagic neoplasm – Irregular lesion with rim-enhancement, “butterfly” appearance, edema, and mass effect.
- Lymphoma – Hyperdense on CT with low ADC values due to hypercellularity, avid enhancement, edema, and mass effect; hemorrhage less common.
- Multiple sclerosis/tumefactive demyelination – T2 hyperintense, may have incomplete/open ring of enhancement, hemorrhage unusual
- Diffuse axonal injury – Post-traumatic, hemorrhagic (microbleeds) + nonhemorrhagic T2 hyperintense foci, involves corticomedullary junction, corpus callosum, deep grey matter and brainstem
- Marchiafava-Bignami – T2 hyperintensity within central body of CC sparing dorsal and ventral periphery (“sandwich sign”), hemorrhage rare.
- Treatment
- Usually conservative. If symptomatic, surgical resection can be considered with caution to preserve venous drainage if associated DVA.
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