Neurosurgery notes/Spine/Spinal vascular diseases/Spinal cavernomas

Spinal cavernomas

View Details
Status
In progress
logo
Parent item
Spinal vascular diseases

General

  • CCM2 - 1303723204
  • Angiographically occult lesions
    • unless associated with a deep venous anomaly (DVA).

Numbers

  • Very rare
    • More often in brain
    • Spinal cord cavernomas are uncommon, accounting for 3-5% of all cavernous malformations.
  • Low - pressure

Uncertain natural hx

  • Haemorrhage risk
    • Spinal CMs: data is limited.
    • Annual haemorrhage rate of 2.1% has been reported.
    • 17% of patients with spinal CMs also have cerebral CMs,
    • 12% of pts had a family history of CMs.

Location

  • Thoracic region (50%)
  • Cervical cord (40%)
  • Uncommonly identified in the conus (10%)

Pathology

  • vascular malformations consisting of closely packed large sinusoid-like vascular channels with little or no intervening nervous tissue.

Subtype

  • Intramedullary cavernoma: Badhiwala et al., 2014 Meta-analysis N=632
    • Cerebral cavernomas and spinal cavernomas coexist in approximately 50% of cases.
    • Location
      • Thoracic cord 55.2%
      • cervical cord 38%
      • cervicothoracic segments 2.4%
      • lumbar 2.1%
      • conus 1.7%
      • thoracolumbar 0.6%
    • The annual rate of haemorrhage: 2.1%.
    • Surgical intervention
      • Indicated: symptomatic lesions
      • Patients who presented acutely had treatment outcomes superior to those with chronic symptoms.
      • More than 80% of patients undergoing surgery had improved or stable symptoms at final follow- up although early postoperative deterioration was common (27%).
      • While intervention was associated with superior outcomes it should be noted that only 10% of patients in these studies were managed conservatively.
  • Extradural cavernoma
    • Rare
    • Occasionally require surgical attention.
    • Purely intraosseous lesions
      • most common
      • rarely become symptomatic.
    • Epidural lesions
      • Clinical features (dependent on their size and location)
        • Radicular pain
        • Myelopathy
    • Surgical resection
      • Mühmer et al., 2014: yields good results in 92% of patients

Investigation

  • MR
    • A well-defined lesion causing focal expansion of the cord with mixed signal intensity on T1WI and T2WI.
    • These lesions are typically surrounded by a complete hypointense rim owing to hemosiderin deposition.
    • No or only sparse spinal cord oedema
    • MRI + C:
      • variable.

Clinical features

  • Sudden onset paraplegia in a young adult.

Treatment

  • Recommended when lesions are symptomatic.
    • The definition of haemorrhage is problematical however in a lesion characterized by slow egress of blood products into surrounding tissues.
    • Should overt haemorrhage be considered a less favourable predictor of subsequent disability than symptomatic microhaemorrhages
  • Radiosurgery and spinal cavernoma
    • Surgical extirpation remains the standard of care when intervention is warranted.
    • SRS less data to support its use.
      • A single case report describes apparently successful treatment of a paraspinal cavernoma after surgery was abandoned because of bleeding (Sohn et al., 2009).
      • Treatment of an intramedullary, mid- thoracic cavernoma has been described but with only 6 months of follow- up taken as evidence of effect (Martin et al., 2012) there remains little evidence to support such an application.