title: Thoracolumbar kyphosis in patients with mucopolysaccharidoses: clinical outcomes and predictive radiographic factors for progression of deformity.
abstract: OBJECTIVE
  Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed non-operatively or operatively in our institution.
  
  
  METHODS
  In all 16 patients were included (eight female: eight male; 50% male), of whom nine had Hurler, five Morquio and two Hunter syndrome. Six patients were treated non-operatively (mean age at presentation of 6.3 years; 0.4 to 12.9); mean kyphotic progression +1.5(o)/year; mean follow-up of 3.1 years (1 to 5.1) and ten patients operatively (mean age at presentation of 4.7 years; 0.9 to 14.4); mean kyphotic progression 10.8(o)/year; mean follow-up of 8.2 years; 4.8 to 11.8) by circumferential arthrodesis with posterior instrumentation in patients with flexible deformities (n = 6).
  
  
  RESULTS
  In the surgical group (mean age at surgery of 6.6 years; 2.4 to 16.8); mean post-operative follow-up of 6.3 years (3.5 to 10.3), mean pre-operative thoracolumbar kyphosis of 74.3(o) (42(o) to 110(o)) was corrected to mean of 28.6(o) (0(o) to 65(o)) post-operatively, relating to a mean deformity correction of 66.9% (31% to 100%). Surgical complications included a deep wound infection treated by early debridement, apical non-union treated by posterior re-grafting, and stable adjacent segment spondylolisthesis managed non-operatively. Thoracolumbar kyphosis > +38(o) at initial presentation was identified as predicting progressively severe deformity with 90% sensitivity and 83% specificity.
  
  
  CONCLUSIONS
  This study demonstrates that severe thoracolumbar kyphosis in patients with MPS can be effectively treated by circumferential arthrodesis. Severity of kyphosis at initial presentation may predict progression of thoracolumbar deformity. Patients with MPS may be particularly susceptible to post-operative complications due to the underlying connective tissue disorder and inherent immunological compromise.
  
  
  CONCLUSIONS
  Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses with thoracolumbar kyphosis managed non-operatively or operatively in our institution.
authors: Royal Hospital for Sick Children, Sciennes Road, Edinburgh, EH9 1LF, UK.
authors: S B
authors: SB
authors: Roberts
authors: Royal Hospital for Sick Children, Sciennes Road, Edinburgh, EH9 1LF, UK.
authors: R
authors: R
authors: Dryden
authors: Scottish National Spine Deformity Centre, Royal Hospital for Sick Children, Sciennes Road, Edinburgh, EH9 1LF, UK.
authors: A I
authors: AI
authors: Tsirikos
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007738:Kyphosis / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications* / Q000000981:diagnostic imaging; D011446:Prospective Studies; D011859:Radiography; D013123:Spinal Fusion / Q000379:methods*; D013904:Thoracic Vertebrae / Q000000981:diagnostic imaging; D016896:Treatment Outcome
keywords: instrumentation; kyphosis; mucopolysaccharidosis; spinal fusion; thoracolumbar; treatment