Anterior spinal artery syndrome in two children with genetic thrombotic disorders.
BACKGROUND
Spinal cord infarction is a well-described, but rare, etiology of myelopathy, especially in children. The most common syndrome, anterior spinal artery syndrome (ASAS), is caused by interruption of blood flow to the anterior spinal artery, producing ischemia in the anterior two-thirds of the cord, with resulting neurologic deficits. Causes of ASAS include aortic disease, thoracolumbar surgery, sepsis, hypotension, and thromboembolic disorders.


METHODS
Case reports of 2 patients.


RESULTS
Two children developed spinal cord infarctions consistent with ASAS, mostly likely caused by previously undiagnosed thrombotic disorders. A child with prothrombin variant experienced acute bilateral lower limb weakness without any preceding event. Magnetic resonance imaging (MRI) revealed increased T2 signal in the anterior cord from midthoracic level to the conus medullaris. A child with protein S deficiency developed lower limb weakness 1 day after a posterior thoracolumbar fusion for idiopathic scoliosis. Computed tomography (CT) myelogram revealed no spinal cord compression. The prothrombin variant mutation is associated with a 2-fold risk of thrombotic events. Individuals with protein S deficiency have an 8-fold increased risk of thrombosis.


CONCLUSIONS
As knowledge of the coagulation pathways grows, it is likely that more patients with spinal cord infarctions will be diagnosed with genetic thrombotic disorders as the etiology of their injury. We review these two disorders, prothrombin variant and protein S deficiency, and the considerations for long-term anticoagulation.
Hakimi
Kevin N
KN
Department of Rehabilitation Medicine, Box 356490, University of Washington, 1959 NE Pacific Street, Seattle, WA 98195-6490, USA. khakimi@u.washington.edu
Massagli
Teresa L
TL
D000293:Adolescent; D020759:Anterior Spinal Artery Syndrome / Q000235:genetics*; D002648:Child; D005260:Female; D006801:Humans; D007020:Hypoprothrombinemias / Q000150:complications*; D008297:Male; D018455:Protein S Deficiency / Q000150:complications*