Congenital pulmonary airway malformation

Case contributed by Dr Alami Badreeddine

Presentation

Recurrent chest infection with progressive respiratory distress

Patient Data

Age: 5
Gender: Male

Chest radiograph shows thin walled cysts with multiple internal septa expand left lung and displace diaphragm and mediastinum. 

CT scan shows replacement of the left lung by cystic lesions of varying sizes.

Case Discussion

Congenital pulmonary airway malformation, formerly known as congenital cystic adenomatoid malformation, is a rare multicystic, intralobar mass of disorganized lung tissue, most often seen in the lower lobes. It is usually diagnosed in children but can be left unrecognized until adulthood.

It can be categorized into three subtypes on the basis of its appearance:

  • Type 1 is most frequent and consists of multiple cysts 2 cm or greater in diameter. On CT, the disease appears as a large, air-filled multicystic lesion.
  • Type 2 is defined as multiple cysts smaller than 2 cm.
  • Type 3, the least frequent type, there are numerous microscopic cysts that often have a more solid appearance on imaging.

Complications of congenital pulmonary airway malformation include infection and malignant transformation. Treatment of congenital cystic lesions usually consists of simple resection of the involved tissue or lobectomy.

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Case information

rID: 28600
Case created: 1st Apr 2014
Last edited: 17th Mar 2016
Systems: Chest, Paediatrics
Inclusion in quiz mode: Included

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