title: Long-term outcome after external tracheal stabilization due to congenital tracheal instability.
abstract: BACKGROUND
  Long-segment tracheobronchial malacia may cause life-threatening dysfunction of the airway system at different levels. This study presents the long-term follow-up (1992 through 2008) of patients who received surgical treatment with external tracheal stabilization in our institution.
  
  
  METHODS
  Eleven patients fulfilled the inclusion criteria. In surviving patients who presented for reexamination, pulmonary function testing, ergometry, and magnetic resonance imaging (MRI) were performed.
  
  
  RESULTS
  All patients could be weaned from the ventilator and discharged. Patients were aged a median 11 months (range, 3 to 48 months) at operation for tracheal compression. Age at follow-up was 9.1 years (range, 0.5 to 16.3 years). Median follow-up was 7.3 years (range, 0.1 to 15.1 years). Postoperatively, 1 patient was lost to follow-up, and 4 died at 2.6 years (range, 0.5 to 6.6 years) of comorbidities. Pulmonary function testing showed a moderate residual airflow restriction, with maximal vital capacity at 75% of normal (range, 45% to 92%). Treadmill exercise testing demonstrated 70% to 89% of the expected normal values for age. Magnetic resonance imaging examination confirmed tracheal patency, but the lumen of the left main bronchus in 2 patients was 50% smaller than on the right. Diaphragmatic motion was normal in all patients.
  
  
  CONCLUSIONS
  Children with congenital tracheal stenosis benefit from external tracheal stabilization. Survival in patients after external tracheal stabilization is significantly influenced by concomitant conditions.
authors.affiliation: Diagnostic and Interventional Radiology, Section of Pediatric Radiology, University Children's Hospital, Im Neuenheimer Feld 430, Heidelberg, Germany. ley@gmx.net
authors.forename: Sebastian
authors.initials: S
authors.lastname: Ley
authors.forename: Tsvetomir
authors.initials: T
authors.lastname: Loukanov
authors.forename: Julia
authors.initials: J
authors.lastname: Ley-Zaporozhan
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Springer
authors.forename: Christian
authors.initials: C
authors.lastname: Sebening
authors.forename: Olaf
authors.initials: O
authors.lastname: Sommerburg
authors.forename: Siegfried
authors.initials: S
authors.lastname: Hagl
authors.forename: Matthias
authors.initials: M
authors.lastname: Gorenflo
mesh_terms: D001999:Bronchoscopy; D002675:Child, Preschool; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011138:Polytetrafluoroethylene; D019736:Prostheses and Implants; D012129:Respiratory Function Tests; D014132:Trachea; D055090:Tracheomalacia / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*
chemical_list: D011138:Polytetrafluoroethylene