Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Cerebral cavernous malformation
Other Resources UpToDate PubMed

Cerebral cavernous malformation

Contributors: Andrea Wasilewski MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Cerebral cavernous malformations (CCMs) are vascular malformations formed by abnormal collections of dilated capillaries within the brain. Their weak walls make them prone to rupturing, resulting in intracerebral hemorrhage. CCMs have no sex predilection and typically present in adulthood. Patients often present with signs of intracranial hemorrhage including headache, seizure, or focal neurologic symptoms such as weakness, vision changes, or aphasia. The hemorrhages are usually small, but location can be critical. Hemorrhages can be recurrent. Nearly 25% of patients with CCMs remain asymptomatic. CCMs often occur sporadically and are typically solitary. Familial CCMs represent approximately 20% of all cases and are caused by genetic mutations in genes CCM1 (KRIT1), CCM2, and CCM3 (PDCD10), inherited in an autosomal dominant fashion and typically presenting with multiple CCMs. Most CCMs occur supratentorially, although lesions can occur within the posterior fossa and spinal cord. CCMs can also occur as a consequence of prior radiation therapy to the brain.

Codes

ICD10CM:
Q28.3 – Other malformations of cerebral vessels

SNOMEDCT:
444869007 – Cavernous hemangioma of brain

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:05/04/2021
Last Updated:05/04/2021
Copyright © 2022 VisualDx®. All rights reserved.
Cerebral cavernous malformation
Print  
A medical illustration showing key findings of Cerebral cavernous malformation : Seizures, Headache, Aphasia, Paralysis, Weakness
Copyright © 2022 VisualDx®. All rights reserved.