From the Guidelines
Cavernomas are clinically significant because they can cause seizures, focal neurological deficits, and hemorrhages, with an annual hemorrhage risk of approximately 3.3% to 4.5%. These vascular malformations, also called cerebral cavernous malformations or cavernous angiomas, are clusters of abnormal blood vessels with thin walls that can occur in the brain or spinal cord 1. Most cavernomas remain asymptomatic and are discovered incidentally on brain imaging. However, their clinical significance emerges when they bleed or compress surrounding neural tissue.
Clinical Presentation
The clinical presentation of cavernomas can vary, with some patients presenting with hemorrhage, seizures, or focal neurological deficits, while others may remain asymptomatic 1. The risk of recurrent hemorrhage from an untreated cavernous malformation is estimated to be 4.5% per year, with higher risks in deeper lesions 1.
Management
Management of cavernomas depends on symptoms, location, and bleeding history. Asymptomatic cavernomas are typically observed with periodic MRI surveillance. For symptomatic cases, anticonvulsant medications may control seizures, and surgical resection is considered for accessible, symptomatic cavernomas, particularly those with recurrent hemorrhages or medically refractory seizures 1. Deep-seated cavernomas in eloquent brain regions may require stereotactic radiosurgery. Patients should avoid anticoagulants and blood thinners when possible, as these may increase bleeding risk.
Key Considerations
- The annual hemorrhage risk for cavernomas is approximately 3.3% to 4.5% 1
- Multiple cavernous malformations have been identified in 13% of sporadic cases and 50% of familial cases 1
- Genetic analysis of families with multiple cavernous malformations has identified mutations of at least 3 genes: CCM1, CCM2, and CCM3 1
- Regular follow-up with neurology or neurosurgery is essential for monitoring growth or changes in symptomatic status.
From the Research
Clinical Significance of Cerebral Cavernous Malformation (Cavernoma)
The clinical significance of cerebral cavernous malformation (cavernoma) can be understood through its various presentations and management strategies.
- Cavernomas are congenital cerebrovascular hamartomas that occur sporadically or familial, comprising multiple sinusoidal spaces filled with blood, without enlarged arterial feeders and draining veins 2.
- They can present with a range of symptoms, including:
- Cross intracranial hemorrhage
- Seizures
- Focal neurological deficits
- Unspecific symptoms like headaches, vertigo, and tinnitus
- The natural course of cavernomas reveals an annual risk of bleeding (0.6%) and a risk of developing epilepsy (1.5%) 2.
- Management strategies for cavernomas include:
- Microsurgical resection, which is the first choice of treatment for symptomatic lesions 2, 3
- Stereotactic radiosurgery, which is only indicated in progressively symptomatic cases that are surgically inaccessible 2, 4
- Conservative management, which involves monitoring patients with regular imaging and medication, and may be considered depending on the patient's symptoms and overall condition 5, 4
Factors Influencing Management Decisions
The management of cavernomas is challenging due to numerous factors that influence decision-making, including:
- Risk stratification of cavernoma-related hemorrhage
- Weighing the risks of surgical intervention against the natural course of the cavernoma
- Clinical presentation and location of the lesion 6, 4
- Size and depth of the cavernoma, with deep-seated cavernomas posing a higher risk of postoperative neurological deficit 2
Treatment Outcomes and Follow-Up
Treatment outcomes for cavernomas can vary depending on the management strategy employed.