title: Long-term clinical and radiological outcomes of Copenhagen syndrome with 19 affected levels: a case report.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
authors.affiliation: The Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, London HA7 4LP, UK. Electronic address: fmalagelada@gmail.com.
authors.forename: Francesc
authors.initials: F
authors.lastname: Malagelada
authors.affiliation: The Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, London HA7 4LP, UK.
authors.forename: Mathew D
authors.initials: MD
authors.lastname: Sewell
authors.affiliation: The Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, London HA7 4LP, UK.
authors.forename: Amr
authors.initials: A
authors.lastname: Fahmy
authors.affiliation: The Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, London HA7 4LP, UK.
authors.forename: Alexander
authors.initials: A
authors.lastname: Gibson
mesh_terms: D005260:Female; D006801:Humans; D007223:Infant; D007738:Kyphosis / Q000151:congenital / Q000175:diagnosis* / Q000000981:diagnostic imaging; D008159:Lumbar Vertebrae / Q000002:abnormalities; D011859:Radiography; D013577:Syndrome; D013904:Thoracic Vertebrae / Q000002:abnormalities
keywords: Copenhagen syndrome; Kyphosis; Magnetic resonance imaging; Outcome; Progressive noninfectious anterior vertebral fusion; Radiology; Spine