From the Guidelines
Cavernomas of the brain are abnormal clusters of blood vessels that form in the brain tissue, consisting of enlarged, thin-walled blood vessels that can range in size from a few millimeters to several centimeters, and can occur anywhere in the brain or spinal cord. These lesions, also called cerebral cavernous malformations (CCMs), appear like a raspberry or mulberry and can be either sporadic (occurring randomly) or familial (inherited) 1. Cavernomas affect approximately 0.5% of the population and can cause symptoms when they bleed, creating pressure on surrounding brain tissue. Common symptoms include seizures, headaches, neurological deficits like weakness or numbness, vision or hearing problems, and in some cases, stroke-like symptoms.
Characteristics of Cavernomas
- They are composed of endothelial spaces containing venous blood, and account for 17% of hemorrhagic stroke in children 1
- They can increase in size over time, probably due to repeated episodes of hemorrhage with subsequent tissue fibrosis and calcification 1
- They have an annual hemorrhage rate of 3.3% to 4.5% 1
- Multiple cavernous malformations have been identified in 13% of sporadic cases and 50% of familial cases, with genetic analysis identifying mutations of at least 3 genes: CCM1, CCM2, and CCM3 1
Diagnosis and Treatment
- Diagnosis typically involves MRI imaging, which shows a characteristic "popcorn" appearance with a dark rim of hemosiderin (blood breakdown products) surrounding the lesion 1
- Treatment depends on the location, size, and symptoms, ranging from observation for asymptomatic lesions to surgical removal for those causing significant problems
- Medications may be prescribed to manage symptoms like seizures, but they don't treat the cavernoma itself
- The risk of bleeding from a cavernoma is approximately 0.7-4% per year, with higher risks for those that have previously bled 1
From the Research
Definition and Characteristics of Cavernomas
- Cavernomas, also known as cerebral cavernous malformations, are the most common vascular malformations and can be found in many locations in the brain 2.
- They are angiographically occult, meaning they are not visible on traditional angiography, and their diagnosis relies on various MR imaging techniques 2.
Clinical Presentation and Symptoms
- If left untreated, cavernomas may lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches 2.
- Seizures are a frequent symptom in patients with cerebral cavernous malformations, and the natural history of seizures can vary depending on the location and characteristics of the lesion 3, 4.
- Patients with cavernomas may experience different types of seizures, including partial seizures, and the frequency and severity of seizures can vary 3.
Treatment and Management
- The clinical presentation and location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas 2.
- Treatment options include microsurgical resection, stereotactic radiosurgery, and conservative management, and the choice of treatment depends on the lesion characteristics and the patient's overall health 2.
- Surgical resection of the cavernoma can be an effective treatment for seizures, especially in patients with a single seizure or rare seizures 3, 5.
- Early surgical resection may offer better outcomes, including seizure freedom and the ability to wean off antiepileptic medications, especially in patients with only one or two preoperative seizures 5.
Special Considerations
- Patients with cavernomas may be at risk of developing psychosis, especially in the setting of hemorrhagic transformation or as a side effect of certain medications, such as levetiracetam 6.
- A thorough pre- and postsurgical evaluation is necessary to determine the best course of treatment and to monitor for potential complications 4.