Anterior spinal artery syndrome in a 13-year-old boy 8 days after taekwondo-fight: vascular obliteration due to vessel lesion or thrombophilia?

BACKGROUND: Spinal cord infarction from anterior spinal cord syndrome (ASAS) in children is a rare pathology and comprises the following clinical symptoms: sudden onset of pain and flaccid para- or tetraparesis, bladder dysfunction, and dissociated sensory loss with impairment of pain and temperature perception. Deep sensibility is not affected. PATIENT: A 13-year-old male patient presented to our emergency department with a bilateral leg weakness. 1 week before, he had suffered a leg strain in a Taekwondo-fight from which he recovered completely. On physical examination our patient's legs were in flaccid paralysis, tone was decreased and he had dissociated sensory loss and acute retention of urine. Blood count, ESR, electrolytes, serologic tests for various pathogens and CSF examination all were normal. However, tests for values of an acute endothelial lesion were increased and he was a homozygous carrier of MTHFR-polymorphism. MRI performed on the day of admission was normal but showed dramatic changes 2 days later with increased signal intensity in the ventral aspect of the spinal cord, characteristic for an ASAS. Treatment included highdose methylprednisolone, a suprapubic bladder catheter, sufficient anticoagulation and a rapid transfer to a rehabilitation centre. DISCUSSION: We assume that a combination of the patient's prothrombotic risk factor (MTHFR-polymorphism with elevated homocysteine levels) and his trauma in the taekwondo-fight with consecutive vessel injury caused an occlusion of the artery by late emboli or a growing thrombus.

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. CONCLUSION: 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.