MYELOPATHY AND MULTIPLE ANEURYSMS ASSOCIATED WITH AORTIC-ARCH INTERRUPTION - CASE-REPORT

WE REPORT A very unusual association of multiple aneurysms in the cran ial cavity and the spinal canal, anterior spinal artery syndrome, syri ngomyelia, and aortic arch interruption between the origins of the lef t 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 uri nary incontinence after two spontaneous subarachnoid hemorrhages was e ncountered in August 1993. Through extensive physical and neuroradiolo gical investigation, an association was found. Because almost all infa nts with aortic arch interruption die within the first month of life, this older patient gave us an opportunity to study the collateral netw ork and the effect of hemodynamic abnormality on the central nervous s ystem. For treatment, we believe that surgery should be directed to th e hemodynamic abnormality of the aortic arch interruption. After bypas s surgery from the ascending aorta to the abdominal aorta with an arti ficial vessel graft, the patient had some neurological improvement, as demonstrated by the sensation level lowering from T7 to L1.