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EDITORIAL
Year : 2014  |  Volume : 62  |  Issue : 4  |  Page : 345--346

Intramedullary spinal cord cavernous angiomas: To treat or not to treat?

Deepak Agrawal1, Sanjay Behari2,  
1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Correspondence Address:
Deepak Agrawal
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi - 110 029
India




How to cite this article:
Agrawal D, Behari S. Intramedullary spinal cord cavernous angiomas: To treat or not to treat?.Neurol India 2014;62:345-346


How to cite this URL:
Agrawal D, Behari S. Intramedullary spinal cord cavernous angiomas: To treat or not to treat?. Neurol India [serial online] 2014 [cited 2021 Jun 24 ];62:345-346
Available from: https://www.neurologyindia.com/text.asp?2014/62/4/345/141172


Full Text

Surgical management of spinal cord cavernous angiomas is formidable due to the attendant risk of additional neurological deficits associated with any surgical endeavour. Surprisingly, the natural history of spinal cord cavernous angiomas is relatively benign. In a study by Kharkar et al., [1] 10 patients with an intramedullary spinal cord cavernous angioma were managed conservatively over an average period of 42 months. The authors found that there was no significant progression in neurological deficits at follow-up despite the presence of pre-existing myelopathy and motor deficits in many of them prior to the start of the study. Pain may also be a presenting feature in a significant number of patients. [2] Due to the potential risk of development of additional neurological deficits, however, the current standard of care is to offer surgery for exophytic/subpial lesions and to keep deep-seated (asymptomatic or mildly symptomatic) lesions under regular observation (unless the patient has recurrent or progressive deficits or presents with sudden neurological deterioration due to an intralesional hemorrhage). [3]

The radiological features of spinal cord cavernous angiomas may often resemble that of other spinal cord pathologies such as demyelinating lesions, transverse myelitis, metastatic disease, hemangioblastomas, and spinal arteriovenous malformations. A hypointense rim around a mixed signal intensity lesion (due to hemosiderin deposits) on T2-weighted MR imaging is pathognomonic of spinal cord cavernous angiomas. Lack of enhancement may also help in differentiating spinal cord cavernous angiomas from other lesions. However, inactive lesions of multiple sclerosis may not enhance and can demonstrate mixed or hyperintense signal intensity. In difficult cases, absence of response to steroids and a stable size on serial magnetic resonance imaging (MRI) may help in differentiating spinal cord cavernous angiomas from demyelinating disorders. [4]

During surgery, an ultrasound is a useful adjunct to localize the lesion accurately, especially in cases where the cavernous angiomas are deep seated. The resection cavity should also be inspected both visually as well as with an ultrasound to ensure completeness of excision.

Intramedullary cavernous malformations may often have an associated venous malformation that should be meticulously preserved in order to prevent development of a venous infarction. [5]

In the article titled "Surgical Management of Intramedullary Cavernous Angiomas and Analysis Pain Relief" by Gong et al., the authors share their experience in treating patients with an intramedullary spinal cord angioma over seven years. [6] Despite having significant limitations that include a retrospective design with a small sample size, the study shows that 60% of patients with intramedullary spinal cord angiomas who are treated surgically will have some pain relief which will be maintained over a follow up of one year. Conversely, nearly 25% of the patients will end up with pain that is worse as compared to baseline levels before surgery. Several patients may have initial pain relief but a lasting cure is often elusive. Occasionally, compromise of the posterior columns during a posterior midline myelotomy may give rise to disabling paresthesias which may heighten the emotional reaction to the already existing pain that the patient may be experiencing. The reaction to pain may vary from person to person and may often be dependent on the patient's personality, emotional support, and cultural differences. These features have not been considered while evaluating the surgical outcome of pain relief.

The findings of this study are in concordance with previous studies that suggest that relief of pain after surgical excision particularly of deeply embedded intramedullary lesions may be transient with a significant number of patients having worsening of pain on being assessed by a numerical pain score after surgery. [2] One interesting aspect of the study was the finding of a more sustained relief of radicular rather than funicular pain. The authors state that this effect could be due to relief of compression on the nerve roots. [1] Another explanation could be that in a deeply situated intramedullary cavernous angioma, segmental involvement of pain fibers may also simulate radicular pain due to the presence of a segmental dermatomal distribution of pain. A cavernous angioma that has bled into the surrounding cord parenchyma would often not have a good plane of cleavage. Attempting complete excision of these lesions, particularly in patients with minimal neurological deficits, would be subjecting them to an inordinate risk of neurological deterioration. [3],[4] The authors have alluded to a rare subset of patients who had a rapid progression in the size of the lesion but were without neurological deficits. [1] These patients were subjected to surgery. In regular clinical practice, this situation is extremely rare and the decision to perform surgery or to treat the patient conservatively under these circumstances is entirely a surgeon's personal choice. All these features raise the doubt whether surgery for excising a deep-seated intramedullary cavernous angioma would actually be of value in patients with funicular pain and without neurological deficits.

These lesions are usually accessed by a midline myelotomy or through the dorsal root entry zone (DREZ). The latter approach appears to be particular alluring as removal of the tumor may automatically entail a microsurgical DREZotomy, which in itself may lead to prolonged pain relief. However, this remains a conjecture as no one has studied this approach for pain relief in intramedullary spinal cord cavernous angiomas until date.

References

1Kharkar S, Shuck J, Conway J, Rigamonti D. The natural history of conservatively managed symptomatic intramedullary spinal cord cavernomas. Neurosurgery 2007;60:865-72.
2Kim LJ, Klopfenstein JD, Zabramski JM, Sonntag VK, Spetzler RF. Analysis of pain resolution after surgical resection of intramedullary spinal cord cavernous malformations. Neurosurgery 2006;58:106-11.
3Deutsch H. Pain outcomes after surgery in patients with intramedullary spinal cord cavernous malformations. Neurosurg Focus 2010;29:E15.
4Gross BA, Du R, Popp AJ, Day AL. Intramedullary spinal cord cavernous malformations. Neurosurg Focus 2010;29:E14.
5Vishteh AG, Sankhla S, Anson JA, Zabramski JM, Spetzler RF. Surgical resection of intramedullary spinal cord cavernous malformations: Delayed complications, long-term outcomes, and association with cryptic venous malformations. Neurosurgery 1997;41:1094-100.
6Gong, et al. ′Surgical Management of Intramedullary Cavernous Angiomas and Analysis Pain Relief Neurol India 2014.