Congenital pulmonary airways malformation

Case contributed by Dr Aliakbar Sahraei

Presentation

the patient is a term Neonate who developed respiratory distress after delivery

Patient Data

Age: 2 days old
Gender: Female

Chest radiography demonstrates a large left sided multicystic (air-filled) lesion with marked mediastinal shift to the right and depression of the left hemidiaphragm. Differentials for this appearance would include CPAM or congenital lobar emphysema. Congenital diaphragmatic hernia thought less likely given the air filled stomach and intestinal loops in the abdomen. 

Selected CT images of the chest confirms a large multicytic lesion which almost entirely occupies the left lung with marked midline shift to the right. Many of the cysts are measure more than 2 cm. This would be in keeping with a large type I CPAM lesion.

Case Discussion

type I of CCAM:

  • most common: 70% of cases
  • large cysts
  • one or more dominant cysts: 2-10 cm in size
  • surrounded by smaller cysts
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Case information

rID: 34607
Case created: 28th Feb 2015
Last edited: 9th Sep 2015
Systems: Paediatrics, Chest
Inclusion in quiz mode: Excluded

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