Cavernoma / cavernous malformation (cavernous angioma)

FACTS

  • low-flow vascular lesions composed of dilated capillary channels without intervening brain tissue.
  • Hemorrhage risk is generally low (~0.5–1%/yr), but increases after a symptomatic bleed, particularly in brainstem lesions.
  • Present with seizures, focal deficits, headache, or acute neurologic change from hemorrhage.
  • Surgery is considered for accessible lesions causing symptoms, recurrent hemorrhage, or medically refractory epilepsy.
  • associated with DVA (developmental venous anomaly)
  • Associations: radiation, familial (AD) associated with multiple lesions, capillary telangiectasias

HPI

  • bleeding: severe HA, altered sensorium
  • chronic: seizures
  • Brainstem symptoms: gait instability, dysphagia, vertigo—important for posterior fossa cavernomas.

PHYSICAL EXAM

universal neuro exam

IMAGING

MRI Brain with and without cavernoma protocol
  • T2: "popcorn" lesions w/ rim of signal loss due to hemosiderin
  • SWI (gradient echo): prominent blooming
  • distinguish from AVM: no contrast enhancement, no feeders

A/P

  • operate only if symptomatic / growing
  • Counsel:
    • usually incidental/asymptomatic, but do have a lifetime risk of seizures, hemorrhage
      • supratentorial & asymptomatic: 4% 5 year hemorrhage / deficit risk
      • infratentorial & asymptomatic: 8% 5 year hemorrhage / deficit risk
      • brainstem / hemorrhaghic lesion: 31% 5 year recurrent hemorrhage risk
    • first bleed rarely fatal, but rebleeds can cause disability cumulatively

Operative cavernoma

most cavernomas are incidental and may seem trivial, but they can also be devastating. This is a CTA negative bleed that was diagnosed as a cavernoma only from a surgical specimen obtained following emergent L crani.
notion image

Familial cavernous malformations

followed AD pattern
mutations in CCM1, CCM2, CCCM3