Spinal cord cavernous malformation
Spinal cord cavernous malformations, also known as spinal cavernomas, are vascular malformations that occur within the spinal cord.
This article specifically relates to spinal cord cavernomas. For a discussion of cerebral cavernomas and a general discussion of the pathology refer to the main article cerebral cavernous malformation.
Spinal cord cavernomas are rare, representing ~5% of intramedullary lesions in adults and 1% of intramedullary lesions in children 3.
Peak presentation is during the fourth decade, which is similar to the peak incidence of cerebral cavernomas 4. Females are more commonly affected than males.
Common presenting symptoms include pain, weakness and paresthesias. Four patterns of clinical presentation have been described 4:
- discrete episodes of neurological deterioration with varying degrees of recovery between episodes
- slow progression of neurological decline
- acute onset of symptoms with rapid decline
- acute onset of mild symptoms with subsequent gradual decline lasting weeks to months
Episodes of haemorrhage have been proposed as the mechanism underlying acute episodes of neurological deterioration. Progressive myelopathy may be caused by micro haemorrhages and gliosis.
Like intracranial cavernomas, spinal cavernomas consist of blood-filled endothelial-lined spaces lined by thickened, hyalinised walls that lack elastic fibres and smooth muscle 5.
More than half of spinal cavernomas are located in the thoracic cord. The second most common location is the cervical cord 4.
- often occult 3
- often occult 3
Minimal cord expansion or oedema unless there has been recent haemorrhage.
- rounded regions of heterogeneous signal intensity on T1 and T2 weighted images due to blood products of varying ages (“popcorn appearance”)
- low signal intensity rim on T2 weighted images (haemosiderin)
- gradient echo (GE): there can be hypointense “blooming” on gradient echo sequences (haemosiderin)
- T1 C+ (Gd): may demonstrate minimal enhancement on post-contrast images
There is minimal cord expansion or oedema unless there has been recent haemorrhage
Treatment and prognosis
Early surgical resection should be considered for all symptomatic patients before repeated haemorrhage or enlargement occur 4.
- 1. Vishteh AG, Sankhla S, Anson JA et-al. Surgical resection of intramedullary spinal cord cavernous malformations: delayed complications, long-term outcomes, and association with cryptic venous malformations. Neurosurgery. 1997;41 (5): 1094-100. Neurosurgery (link) - Pubmed citation
- 2. Labauge P, Bouly S, Parker F et-al. Outcome in 53 patients with spinal cord cavernomas. Surg Neurol. 2008;70 (2): 176-81. doi:10.1016/j.surneu.2007.06.039 - Pubmed citation
- 3. Kharkar S, Shuck J, Conway J et-al. The natural history of conservatively managed symptomatic intramedullary spinal cord cavernomas. Neurosurgery. 2007;60 (5): 865-72. doi:10.1227/01.NEU.0000255437.36742.15 - Pubmed citation
- 4. Ogilvy CS, Louis DN, Ojemann RG. Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management. Neurosurgery. 1992;31 (2): 219-29. Neurosurgery (link) - Pubmed citation
- 5.Osborn AG. Diagnostic neuroradiology. Mosby Inc. (1994) ISBN:0801674867. Read it at Google Books - Find it at Amazon
- 6. Grossman RI, Yousem DM. Neuroradiology, the requisites. Mosby Inc. (2003) ISBN:032300508X. Read it at Google Books - Find it at Amazon