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Cavernoma

Summary

  • Cavernomas are vascular malformations composed of abnormal dilated blood vessels
  • Typically found in the brain and spinal cord, they can cause seizures, haemorrhage, and neurological deficits
  • Characteristically have a "popcorn" or "mulberry" appearance on T2-weighted imaging and hypointense on SWI

Pathophysiology

  • Composed of closely packed, thin-walled capillary-like channels (caverns) lined by a single layer of endothelium
  • Lack intervening brain parenchyma between vascular spaces
  • Sporadic cases are more common, but familial forms exist (CCM1, CCM2, CCM3 genes)
  • May be a delayed complication of radiation therapy
  • Often asymptomatic but may cause seizures or neurological deficits after haemorrhage

Demographics

  • Prevalence: 0.4-0.9% in the general population
  • Can occur at any age, but most commonly diagnosed in young adults (20-40 years)
  • No significant gender predilection

Diagnosis

  • Often asymptomatic and discovered incidentally on imaging
  • Can be difficult to identify if obsured by an acute haematoma
  • Can occur alongside a developmental venous anomaly (~33% of cases)

Imaging

  • CT

    • Potentially hyperdense due to calcification or acute haemorrhage

  • MRI

    • T2 "Popcorn" or "mulberry" appearance with mixed signal intensity
    • SWI Hypointense due to haemosiderin deposition and potentially calcification
    • T1 Variable depending on presence or age of blood product
    • ++T1+C++ Absent (or minimal) enhancement

  • Catheter angiogram

    • Not visible on DSA ("angiographically occult")

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  • 20 years prior, the patient presented with right sided sensory disturbance.
  • A large cavernoma in the left thalamus was stable on longterm follow-up.

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  • A 40-year-old patient presented with a headache and visual disturance.
  • CT showed an acute haematoma
  • MRI showed mixed high and low T2-weighted signal.
  • No abnormality was identifed on CTA, MRA or DSA.

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  • A 45-year-old patient presented with paraesthesia in both hands.
  • MRI showed a classic "popcorn" texture lesion with a rim of haemosiderin with no evidence of acute haemorrhage

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  • A 30-year-old patient with temporal lobe epilepsy.
  • Right temporal lobe cavernoma with central "popcorn" hyperintensity and a rim of haemosiderin staining.

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  • A 30-year-old presented with left-sided facial weakness and numbness.
  • The initial MRI showed a T1- and T2-hyperintense lesion in the left side of the pontine tegmentum, consistent with a recent haemorrhage within a cavernoma. SWI showed a network of small vessels representing an associated development venous anomaly
  • 6 months later, mild rim of oedema on FLAIR, blooming artefact and T1-hyperintensity had regressed.

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  • A 25-year-old patient was treated with whole brain and spine radiotherapy due to a disseminated medulloblastoma.
  • An MRI performed 10 years after treatment, showed extensive radiation induced changes including hazy white matter hyperintensity, multiple cavernomas (red arrow), microhaemorrhages and excessive mineralisation in the basal ganglia.
  • The phase data from SWI showed mixed paramagnetic haemosiderin (black) and diamagnetic calcium (white).

panels-1 * A 40-year-old patient was under surveillence for a pontine cavernoma. * The patient presented acutely with headache, ataxia and diplopia. * An MRI on admission showed enlargement of the pontine cavernoma with T1 shortening consistent with acute haemorrhage.

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Treatment

  • Observation:
    • Asymptomatic lesions or those in eloquent brain areas
    • Regular MRI follow-up to assess for haemorrhage or growth
  • Surgical resection:
    • Considered for symptomatic lesions (especially those causing seizures or repeated haemorrhage)
    • Complete resection is curative
  • Stereotactic radiosurgery:
    • Alternative for deep-seated/surgically inaccessible lesions
    • Can reduce haemorrhage risk but does not eliminate it
  • Antiepileptic drugs:
    • For seizure control in patients with epilepsy
  • Genetic counseling:
    • For familial cases to discuss inheritance patterns and screening of family members

Differential diagnosis

Differential Diagnosis Distinguishing Feature
Capillary telangiectasia Typically smaller; lacks hemosiderin rim on MRI; classically in the pons; may show faint enhancement
Developmental venous anomaly Can occur alongside a cavernoma; linear vascular flow void
Arteriovenous malformation Flow voids on MRI; angiographic blush
Hemorrhagic metastasis Multiple lesions; surrounding vasogenic oedema; identification of primary tumour
* A 40-year-old patient presented 2 years prior with headache.
* Initial imaging (not shown) showed a haematoma in the left insular region.
* MRI showed many other low intensity lesions within the brain and cervical cord representing cavernomas.
* The T1-shortening within the frontal operculum represented evolving blood product from the haemorrhage at presentation.