Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 58, Num. 4, 2010, pp. 675-676

Neurology India, Vol. 58, No. 4, July-August, 2010, pp. 675-676

Letter To Editor

Hematomyelia due to anterior spinal artery aneurysm in a patient with coarctation of aorta

Sharma Sandeep, Kumar Subhash

Department of Neuroradiology, AIIMS, New Delhi

Correspondence Address:Department of Neuroradiology, AIIMS, New Delhi, drsharma_sandeep@yahoo.com

Date of Acceptance: 31-Jan-2010

Code Number: ni10184

PMID: 20739828

DOI: 10.4103/0028-3886.68697

Sir,

Coarctation of aorta causes multiple enlarged collaterals which shunt blood from the supra to infra coarctation segment of aorta and anterior spinal artery (ASA) is one such route. Such collaterals can result in compressive myelopathy. However, hematomyelia secondary to aneurysmal dilatation of the ASA is distinctly rare.

A 36-year-old male presented to the department of neuroradiology with sudden onset weakness of all four limbs associated with loss of sensation of below the neck and urinary and fecal incontinence of two months duration. On examination he had 0/5 motor power in the lower limbs and 3/5 power in the upper limbs and sensory loss below C6 level Deep tendon reflexes were brisk and plantars were bilaterally up going. Magnetic resonance images (MRI) [Figure - 1]a to d showed hematomyelia [Figure - 1]a to d extending into the subarachnoid space (not seen in the image). Digital subtraction angiography (DSA) [Figure - 2] a to c showed coarctation of aorta and multiple collaterals bypassing the narrowing to supply the descending aorta from branches of both subclavian arteries. There was a large radiculomedullary artery from the right thyrocervical trunk supplying the anterior spinal artery with an aneurysm of ASA at C6 level [Figure - 2]b and c. Selective injection of this artery showed contrast flowing retrogradely through a lower dorsal segmental artery into the abdominal aorta [Figure - 2]b and c. Embolization was attempted but failed due to technical reasons. Aneurysm was exposed surgically and clipped. Patient made a gradual recovery and his lower limb power improved to 4/5 while his upper limb power and bowel bladder control returned to near normal at four months of follow-up.

The incidence of coarctation of aorta is 0.3 to 0.4/1000 live births. The clinical presenting features depend on the age and severity of narrowing. Most of the patients survive to adulthood and the diagnosis may be incidental. ^[1] About 25% of patients with untreated aortic coarctation, however, die of cardiac failure. The neurological manifestation include both ischemic and hemorrhagic strokes. Coarctation of aorta is often associated with intracranial aneurysms. However, aneurysms of spinal artery are very rare and occur as a result of chronic hemodynamic stress of shunting of blood flow into the descending aorta. Such hemodynamic forces in the spinal artery generally result in engorgement of the vessels in the tight bony canal and consequent cord compression. ^[2] The first angiographic demonstration of enlarged ASA in patients with coarctation of aorta was by Doppman and colleagues. ^[3] These authors reviewed the aortographic features in 40 patients with coarctaion of aorta and found an enlarged trortuous ASA at the cervico-thoracic level in seven patients. Only four case of spinal artery aneurysms in association with coarctation have been reported. ^[2]

Hematomyelia is more commonly due to trauma. Non-traumatic causes include vascular anomalies, tumors, bleeding disorders, drug abuse, syphilis, hemorrhagic myelitis, and aortic aneurysms. ^[4] This case illustrates that hematomyelia can be the initial presenting feature of patients with coarctation of aorta and is due to hemodynamic flow-related spinal artery aneurysm. Aortogram and extensive spinal angiogram is advisable to investigate the cause of hematomyelia in patients with coartication of aorta.

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