Myelopathy and multiple aneurysms associated with aortic arch interruption: case report

Abstract

We report a very unusual association of multiple aneurysms in the cranial cavity and the spinal canal, anterior spinal artery syndrome, syringomyelia, and aortic arch interruption between the origins of the left common carotid artery and the left subclavian artery. A 14-year-old boy with a history of 2 years of flaccid paraplegia and rectal and urinary incontinence after two spontaneous subarachnoid hemorrhages was encountered in August 1993. Through extensive physical and neuroradiological investigation, an association was found. Because almost all infants with aortic arch interruption die within the first month of life, this older patient gave us an opportunity to study the collateral network and the effect of hemodynamic abnormality on the central nervous system. For treatment, we believe that surgery should be directed to the hemodynamic abnormality of the aortic arch interruption. After bypass surgery from the ascending aorta to the abdominal aorta with an artificial vessel graft, the patient had some neurological improvement, as demonstrated by the sensation level lowering from T7 to L1.