Anterior spinal artery syndrome in pediatric patients: diagnostic and therapy

A. M. Cseh, J. Kirschner, H. Meinicke, H. Fuchs, B. Zieger (Freiburg, Germany)

Pediatric and neonatal thrombosis and hemostasis
Date: 17.02.2017,
Time: 17:15 - 18:15

Objective: Infarction of the anterior spinal artery is a rare but severe disease in children. Symptoms comprise sudden onset of flaccid para- or tetraparesis, dissociated sensory loss off pain and temperature perception and bladder dysfunction. The pathophysiology is often associated with vascular lesions with or without previous surgery. Here we present three patients after trauma. Literature is scarce and not conclusive whether therapeutic heparinization is of benefit regarding the long term outcome of these patients.

Methods: We analyzed clinical and laboratory data of three adolescents who were treated in our center with anterior spinal artery syndrome (ASAS). Patient 1 was a 13-year-old boy who developed ASAS a few days after a taekwondo-fight. Patient 2 was a 14-year-old girl who presented eight hours after experiencing a kink during hair-drying. Patient 3, a 15-year-old boy, was admitted one day following a kink during fitness studio strength training. All three patients were found to be homozygous carriers of methylenetetrahydrofolate reductase (MTHFR) A222V-polymorphism. Accordingly, patient 1 and 2 had slightly elevated homocysteine levels. No other thrombophilic factors were detected. They received anticoagulation therapy with unfractionated or low molecular weight heparin, initially in therapeutic later in prophylactic dose. Patient 1 and 2 received heparin for 16 months and 13 months, respectively. Patient 3 has been treated for 3 weeks so far.

Results: Patient 1 and 2 experienced a slow improvement of their symptoms. Patient 1 has an unrestricted quality of life 7 years after the diagnosis. Patient 2 regained motoric skills without a relevant improvement of sensory loss and neurogenic bladder dysfunction within 7 months. Patient 3 is still in the acute phase of ASAS.

Conclusion: Thromboembolic events represent a rare disease category in children. They are significantly associated with congenital or acquired thrombophilic factors. Our patients were carriers for MTHFR-A222V-polymorphism. However, it remains unclear whether this polymorphism plays a crucial role in the pathogenesis of ASAS. Heparin was promptly administered in all cases. Our patients┬┤ outcome seems to be better than the outcome of patients who have been described in the literature. This supports the hypothesis that an early and adequate therapy with heparin improves the clinical outcome.