Presentation
Respiratory distress since birth
Patient Data
Age: 1 month
Gender: Female
From the case:
Pulmonary agenesis
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Info
- absent right lung parenchyma and right main bronchus
- hyperinflation of left lung parenchyma and herniation to right hemithorax
- heart and mediastinal structures in right haemothorax
- absent right pulmonary artery
- normal position and route of IVC
- left sided aortic arch
Case Discussion
The results above reveal congenital right pulmonary agenesis.
Pulmonary agenesis is the entire absence of pulmonary parenchyma, bronchi after bifurcation, and their ancillary arteries. Is an uncommon type of unilateral congenital lung anomaly. Patients typically experience breathing problems as newborns and they almost never go unnoticed until puberty.
Pulmonary agenesis differs from other congenital lung anomalies in that:
- in pulmonary aplasia, it's common to see small, blind-ending rudimentary bronchus.
- pulmonary hypoplasia is defined by a decrease in lung size and weight, as well as a lack of development of some lung tissue, despite the presence of bronchi and alveoli.
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