Neurosurgery notes/Vascular/Non AV shunt/Capillary telangiectasia

Capillary telangiectasia

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Non AV shunt

General

  • Others: AVM, venous angiomas, cavernous malformations, mixed or unclassified angiomas
  • A type of AOVM (angiographically occult vascular malformation)

Definition

  • Aggregation of capillary-type dilated capillaries with normal intervening brain tissue that has low flow
    • without smooth muscle or elastic fibres in their walls
      • lined with a single benign endothelial cell layer
    • If there is no intervening brain tissue it will cavernoma

Numbers

  • 2nd most common vascular malformation affecting brain (1st AVM)
  • Rare
  • affecting ≈ 1 in 5,000 people
  • Prevalence of 0.4-0.7 percent in the general population.

Histopathology

Macroscopic

  • Capillary telangiectasias are small vascular lesions (mm to 20 mm) that do not exert mass effect.

Microscopic

  • Capillary telangiectasias show localized aggregations of thin-walled dilated vessels without smooth muscle or elastic fibres, situated within brain parenchyma lacking adjacent gliosis or calcification.

Clinical presentation

  • Incidentally found without clinical significance
  • Risk of haemorrhage is very low, except possibly in brainstem
  • Usually solitary, but may be multiple when seen as a part of a syndrome:
    • Louis-Barr (ataxia telangiectasia)
    • Myburn-Mason
    • Sturge-Weber
  • Brain capillary telangiectasia is not a part of hereditary haemorrhagic telangiectasia (HHT).
    • HHT is an autosomal dominant neurocutaneous disorder characterized by mucocutaneous capillary telangiectasia and arteriovenous malformations and fistulae in lungs, liver, brain, and spine

Pathophysiology

  • Capillary telangiectasias may be congenital due to failure of capillary involution or acquired via reactive angiogenesis from insults like irradiation or venous hypertension.
    • Autosomal dominant

Location

  • Pons: most common
    • Can also affect the middle cerebellar peduncle, cerebral hemispheres, and spinal cord.

Imaging

  • Usual multiple and incidentally found
  • Can found together with Cavernomas
  • CT
    • Well demarcated homogeneous
    • Mottled high density: due to bleeding, calcification
    • A bit contrast enhancing
    • Rare to have oedema or mass effect
  • MRI
    • T1 C+: hyper intense
        • notion image
    • T2:
      • reticulated core of inc., and dec. intensity,
        • if these was previous haemorrhage can have a rim of hemosiderin laden macrophages that comes up as hypointensity on T2
    • SWI
      • they can be seen as multiple hypointense foci.

Management

  • Surgical indication:
    • recurrent haemorrhage,
    • medical intractable seizure
  • SRS has not had a satisfactory high enough benefit to risk ratio