Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 59, Num. 2, 2011, pp. 317-318

Neurology India, Vol. 59, No. 2, March-April, 2011, pp. 317-318

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A case of aortic coarctation presenting with quadriparesis due to dilated tortuous anterior spinal artery

Maneet Gill¹, Harish Chandra Pathak¹, Prakash Singh¹, Kamal Pathak²

¹ Department of Neurosurgery, Army Hospital (Research and Referral), New Delhi, India
² Department of Intervention Radiology, Army Hospital (Research and Referral), New Delhi, India

Correspondence Address: Maneet Gill, Department of Neurosurgery, Army Hospital (Research & Referral ), Street : Gurgaon Road , Dhaula Kuan, Delhi Cantt, New Delhi - 110010, India, maneet5@yahoo.com

Code Number: ni11094

PMID: 21483153

DOI: 10.4103/0028-3886.79174

A 30year-old male presented with sudden onset weakness of all four limbs. Clinically, he had hypotonia in all the four limbs and motor weakness was graded at 4/5. Deep tendon reflexes were bilaterally brisk and plantar response was extensor. Magnetic resonance imaging of cervical spine [Figure - 1] showed multiple, well--defined, rounded areas of flow void, suggestive of blood vessels in the anterior epidural space from C5 to T3 level. Spinal digital subtraction angiography [Figure - 2] did not reveal any dural arterio-venous fistula. Instead, it revealed coarctation of aorta with prominent collaterals. Computed tomography (CT) angiogram showed dilated vessels connecting the tortuous anterior spinal collateral to extra spinal channels, i.e., the vertebral and the intercostal arteries [Figure - 3]. He showed gradual recovery from his neurological deficits. Surgical correction of coarctation of aorta is being planned.

In patients with coarctation of aorta, spinal complications are often due to aortic cross-clamping during surgery for the correction of the coarctation. However, myelopathy rarely can be the presenting feature of coarctation of aorta. ^[1],[2],[3] In coarctation of aorta, several mechanisms can lead to myelopathy: compression due to dilated intraspinal collaterals, ^[1] subarachnoid hemorrhage and its sequalae, ^[4] compression due to radicular artery aneurysm, ^[5] and spinal artery aneurysm. ^[6] However, myelopathy due to a sufficiently enlarged and tortuous anterior spinal artery collateral is extremely rare,; only 14 such cases have been documented in the English literature. ^[1],[2],[3]

The main collaterals in coarctation are through the internal mammary and the intercostal arteries. In patients with tortuous anterior spinal artery, the myelopathy is not congestive as inspinal dural arterio-venous fistulae. The mechanisms proposed by which a dilated anterior spinal artery can cause myelopathy include: (1) enlarged spinal arteries can cause compressive myelopathy given the space constraint in the tight spinal canal in the lower cervical and upper thoracic regions; (2) "aortic ,steal", ^[3], this mechanism probably explains the reversible nature of the neurological deficits as seen in our patient; and (3) dilated collaterals may serve as sources for clots and throw microemboli occluding the anterior spinal artery, resulting in sudden onset deficits. ^[7] The blood supply to the lower cervical and upper thoracic cord is rather tenuous and depends heavily on the supplementation of the anterior spinal artery by segmental radicular arteries. Between two adjacent radicular arteries, the blood flow is bidirectional, and due to the convergent nature of the flow, there are watershed areas.Detailed clinical angiographic correlation has shown that in cases of coarctation of aorta, there is reversal of flow and the blood passes from the anterior spinal artery, via huge dilated communicating extraspinal vessels to the part of aorta distal to the coarctation. It is as if the post-stenotic aorta were stealing the blood from the anterior spinal artery.

Treatment has to be individualized taking into consideration whether the neurological deficit is stable, improving or worsening. Most patients recover after the surgical correction of the coarctation and the consequent normalization of the blood flow patterns. ^[2],[3] Some patients also recover without the correction of the coarctation, ^[2] as seen in our patient. Tan et al.^[1] have reported regression of symptoms after decompressive laminectomy.

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