Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
October 2017
Next Case of the Month coming November 7...
Cavernous Malformation Presenting with Massive Hemorrhage
- Background:
- Cerebral cavernous malformations (CCMs) are dysplastic collections of dilated, thin-walled blood vessels, grossly described as berrylike clusters, lined by leaky endothelial gap junctions and, in many cases, a distinct hemosiderin rim.
- CCMs may be found anywhere in the body and are often clinically silent unless the brain is involved, in which case the risk of hemorrhage can be life-threatening.
- Four types can be differentiated by MR signal characteristics using the Zabramski classification, with type II (popcornlike lesions) being the most common.
- Other terms for CCM include cavernous hemangioma, cavernous angioma, and cavernoma; however, these are misnomers, as CCMs are not neoplastic.
- Clinical Presentation:
- Common presenting neurologic sequelae include headache, hemorrhage, seizure, hydrocephalus, and strokelike deficits; however, most people are asymptomatic.
- Risk of hemorrhage is greater in familial cases compared with sporadic cases
- Key Diagnostic Features:
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MRI is the optimal modality for diagnosing all types of CCM, although some lesions may be visualized on CT with contrast.
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T1- and T2-weighted sequences demonstrate characteristic berrylike or popcornlike patterns, with or without a blooming rim of hemosiderin.
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GRE T2* and SWI sequences are arguably best at delineating certain lesion types.
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Angiography is not particularly useful, as CCMs exhibit minimal blood flow.
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Calcifications, and even ossifications, may be present in larger lesions.
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- Differential Diagnoses:
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Developmental venous anomaly: common incidental finding that carries risk of venous infarct and frequently occurs simultaneously with CCM; however, these lesions are generally distinguishable by their restriction to certain vein territories.
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Cerebral arteriovenous malformations: should see a nidus arteriovenous shunting on angiography
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Hemorrhagic metastases, particularly melanoma: similar appearance on MRI
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Hemorrhagic primary brain tumors, especially low-grade gliomas
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Cerebral amyloid angiopathy
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Foci of hemosiderin staining seen following whole-brain radiation: punctate hypointense foci on GRE or SWI can mimic type IV CCMs; should not see intrinsic T1 shortening in these lesions
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- Treatment:
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In asymptomatic CCM, expectant management and serial imaging is advised.
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For patients with symptoms, therapeutic options include stereotactic radiosurgery, microsurgical resection, and conservative management.
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