Progressive non-infectious anterior vertebral fusion (Copenhagen Syndrome) in three children: features on radiographs and MR imaging

Abstract

Progressive non-infectious anterior vertebral fusion is a rare childhood disease of unknown aetiology. It commonly presents with kyphosis associated with progressive fusion in the thoracolumbar spine. MR imaging features have not previously been described to our knowledge. Three cases presenting to our institution are described. All three children had other congenital or developmental problems. Plain radiographs showed typical features with narrowing and irregularity of the anterior endplates and developing anterior vertebral fusion. MR imaging was available in all (in one case sequentially at ages 1 and 12 years), and features demonstrated were anterior disc narrowing and anterior endplate irregularity progressing to fusion. Associated bone oedema or fatty replacement was seen in the endplates, and was most marked at the anterior corners.