Cerebral cavernous malformations (CCMs) are the most common vascular malformations of the CNS.
They are clusters of abnormal, thin-walled blood vessels that may bleed, sometimes extending beyond their fragile walls.
Prevalence: ~0.1–0.5% of the population; some have a genetic basis.
More common in Hispanic communities, especially Mexican-Americans.

🔬 Pathophysiology

CCMs lack large feeding arteries or draining veins (“angiographically occult”).
Can occur anywhere in the brain or spinal cord; deeper lesions (e.g. brainstem) bleed more often than superficial ones.
Genetic variants:
- CCM1 → chromosome 7q.
- CCM2 → chromosome 7p.
- CCM3 → chromosome 3p (often more severe, early onset).

🩺 Clinical Presentation

Many are asymptomatic, found incidentally on MRI.
When symptomatic → intracerebral haemorrhage, seizures, focal deficits, raised ICP, headaches, or hydrocephalus.

📍 Cavernomas in the Midbrain

Brainstem cavernomas are particularly high risk due to eloquent location and frequent haemorrhage-related morbidity.

Midbrain Cavernoma Image 1 Midbrain Cavernoma Image 2

🧾 Investigations

CT/MRI: Characteristic “raspberry” or “popcorn-like” appearance, with or without haemorrhage.
MRI SWI/GRE: Best for detecting blood products (haemosiderin). Highly sensitive.
Angiography: Typically negative (lesions are angiographically occult).

📊 MRI Types (Zabramski classification)

🟥 Type I: Subacute haemorrhage → hyperintense core on T1/T2.
🟧 Type II: “Popcorn” lesion → mixed signals on T1/T2 with hypointense rim.
🟨 Type III: Chronic/resolved haemorrhage → isointense core, often familial.
🟩 Type IV: Tiny foci, only visible on GRE/SWI, may mimic capillary telangiectasias.

⚖️ Management

Options: Conservative, microsurgical resection, or stereotactic radiosurgery.
Surgery indicated: Recurrent haemorrhage, intractable seizures, or progressive neurological deficit (esp. non-eloquent regions).
Brainstem cavernomas → controversial but some evidence supports resection due to high morbidity if left untreated.
Conservative management: Annual MRI follow-up if asymptomatic or low-risk.

📖 References

Mouchtouris N. et al., Management of Cerebral Cavernous Malformations: From Diagnosis to Treatment

💡 Exam Pearl: Cavernomas are angiographically occult. Think “popcorn lesion” on MRI → consider cavernoma.

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Cavernous angiomas (Cavernomas)

🧠 Introduction