Fact-sheet: Bronchial atresia


Updated on 10/03/2018 at 9:40 AM

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Definition

Rare congenital malformation with a developmental defect of a lobar or segmental bronchus, with normal growth of the pulmonary tree and vasculature distal to the defect.

Focal obliteration with dilated distal bronchi, mucoid impactions, then bronchocele.

Inflation of the distal parenchyma by collateral ventilation from the adjacent parenchyma through the pores of Kohn and the canals of Lambert.

  • Canals of Lambert: bronchoalveolar communication (30µ).
  • Pore of Kohn: interalveolar communication (<3µ).

Clinical features

  • Asymptomatic for several years
  • Recurrent infectious complications

Radiography

Systematized hyperlucency with ipsilateral hilar opacity (= bronchocele)

CT

  • Parenchymal hypodensity related to expiratory air trapping, in the same location as the bronchocele(s).
  • Bronchocele

Management

  • Favorable prognosis.
  • No treatment and follow-up if asymptomatic.
  • Lobar or segmental resection if infectious complications occur