Long-term clinical and radiological outcomes of Copenhagen syndrome with 19 affected levels: a case report

BACKGROUND CONTEXT: Copenhagen syndrome, or progressive noninfectious anterior vertebral fusion, is a rare disorder of unknown etiology that usually presents with thoracolumbar kyphosis in childhood. There have been no long-term reports on outcome in children with multiple affected levels with longitudinal imaging from infancy to adulthood. PURPOSE: The purpose of this study was to report the long-term outcome of nonoperative management of a child with Copenhagen syndrome affecting 19 vertebral levels. STUDY DESIGN: This study is a case report. METHODS: The study included longitudinal clinical and radiological follow-ups. RESULTS: A 1-year-old female presented with thoracolumbar kyphosis. Plain radiographs and magnetic resonance imaging demonstrated kyphosis associated with anterior disc space narrowing plus T11-T12 and L2-L3 vertebral end-plate abnormalities. Initial treatment with a plaster jacket followed by brace failed to prevent progressive vertebral involvement and kyphosis during childhood. At skeletal maturity, no further levels became involved, and progression was halted. In total, 19 levels showed anterior fusion. CONCLUSIONS: This case describes the long-term outcome of nonoperative management for progressive noninfectious anterior vertebral fusion affecting multiple levels. Extensive vertebral involvement does not always require surgical intervention. There is a need for future research on the prognostic indicators for progression and long-term outcome. (C) 2015 Elsevier Inc. All rights reserved.