Vanishing bronchus intermedius syndrome in a pediatric patient with cystic fibrosis after lung transplantation

Pediatr Transplant. 2012 Dec;16(8):E333-7. doi: 10.1111/j.1399-3046.2012.01682.x. Epub 2012 Apr 11.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Aspergillus fumigatus / metabolism
  • Bronchi / pathology
  • Bronchial Diseases / complications
  • Bronchial Diseases / diagnosis*
  • Constriction, Pathologic
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / etiology*
  • Dilatation
  • Homozygote
  • Humans
  • Lung Transplantation / adverse effects
  • Lung Transplantation / methods*
  • Male
  • Spirometry / methods
  • Stents
  • Syndrome