SURGICAL-MANAGEMENT OF CERVICOMEDULLARY COMPRESSION IN ACHONDROPLASIA

Achondroplasia is often associated with cervicomedullary dysfunction. MRI sagittal imaging was performed to determine whether surgical inter vention was necessary to relieve cervicomedullary compression. Cervico medullary compression was classified into severe and mild types based on the ratios of the brain stem diameter at the foramen magnum, the si te of the most severe stenosis to the diameter at the pontomedullary j unction and C3 level, where it is normal, This classification was foun d to be closely correlated with the severity of clinical symptoms and important in determining whether surgical intervention is necessary. M oreover, since the incidence of sudden death is high in patients aged 4 years or younger with achondroplasia, surgical intervention should b e considered in all such patients if cervicomedullary compression is p resent.