Pulmonary agenesis and respiratory failure in childhood

Abstract

Pulmonary agenesis (PA) is a rare congenital anomaly, which may be unilateral or bilateral. Unilateral PA may be associated with nonspecific respiratory symptoms. We report the case of 5-month-old infant who presented a normal development until the age of 4 months when a respiratory infection caused an acute respiratory distress syndrome with a fatal outcome. The autopsy findings depicted the right lung agenesis without any other concomitant malformation. Although respiratory symptoms represent frequent complaints in pediatrics, the aim of this study is not only to draw attention to the unilateral pulmonary agenesis as a possible underlying malformation in children who present recurrent and severe respiratory symptoms, but also to report a case diagnosed at autopsy.

Keywords: Autopsy; Lung agenesis; Respiratory Insufficiency.

Figure 1. Chest x-ray showing a right hemithorax opacification and left lung vicariance. Note the rightward deviation of the mediastinal structures.

Figure 2. A - Front view of the thoracic organs. Note a single pulmonary artery perfusing the left lung and lack of the right lung. B - Posterior view characterized by the complete absence of the right main bronchus and lung.

Figure 3. A - Non-bifurcate trunk of the pulmonary artery. B - Note at the tip of the forceps the obliterated ductus arteriosus.

Figure 4. Photomicrography of the lung. A - Interstitial inflammatory infiltration and alveolar edema (H&E, 400X). B - The presence of numerous macrophages and edema in the alveolar spaces (Masson’s Trichrome, 400X).