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Keywords:

  • vanishing bronchus intermedius syndrome;
  • cystic fibrosis;
  • lung transplantation;
  • Aspergillus fumigatus

Hayes D Jr, Mansour HM. Vanishing bronchus intermedius syndrome in a pediatric patient with cystic fibrosis after lung transplantation.

Abstract:  Airway complications occur frequently after lung transplantation. Bronchial stenosis is the most frequently encountered complication with the most severe form of that being the vanishing bronchus intermedius syndrome (VBIS). This rare disorder has never been reported in the pediatric population. This is the first report of VBIS in a pediatric patient, specifically a 16-yr-old male patient with cystic fibrosis whose course was complicated by a lower airway infection with Aspergillus fumigatus. The VBIS responded to bronchoscopic balloon dilation and placement of an airway stent.