Congenital pulmonary airway malformation

Case contributed by Dr Hidayatullah Hamidi

Presentation

Respiratory distress since birth.

Patient Data

Age: 40 days
Gender: Male

Large, multi-loculated air-filled, cystic lesion in the right hemithorax resulting in contralateral mediastinal shift to the left. The overall size of the lesion measures 8 x 9 x 7 cm. The locules of the lesion are larger than 2 cm in size. Right sided chest tube is seen with mild pneumothorax and surgical emphysema in the right upper anterior chest wall. 

Evidence of segmental collapse and consolidation in the left lung.

Diaphragm is intact: no evidence of congenital diaphragmatic hernia.

Case Discussion

The features are suggestive of congenital pulmonary airway malformation (CPAM) or congenital cystic adenomatoid malformation (CCAM) type I as the cysts are larger than 2 cm in size.

The chest tube may be inserted by the primary clinician suspicious of pneumothorax as the chest radiographic features of CPAM can be similar, or a real pneumothorax that has also occurred (unclear in this case).

Segmental collapse and consolidation in the left lung probably due to a secondary infectious process.

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Case information

rID: 56681
Case created: 16th Nov 2017
Last edited: 29th Nov 2017
Systems: Chest, Paediatrics
Inclusion in quiz mode: Included
Institution: French Medical Institute for Mothers and Children

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