IS Case 89: Lung Agenesis
Imaging Sciences URMC 2008
Publication Date: 2009-05-20
Pulmonary agenesis was first described by De Pozze in 1673 after he discovered the anomaly at autopsy. Pulmonary agenesis is a rare congenital malformation consisting of total absence of pulmonary parenchyma, blood vessels, and bronchi on the affected side. Pulmonary agenesis is classified morphologically by the extent to which bronchopulmonary tissue is absent. Types include bilateral complete agenesis, unilateral agenesis with (a) complete absence of bronchi, (b) rudimentary bronchus present but no pulmonary tissue, or (c) poorly developed main bronchus with poorly organized parenchyma, and lobar agenesis.
Bilateral pulmonary agenesis is extremely rare and is not compatible with extrauterine life. Unilateral pulmonary agenesis does not result in death and is compatible with long-term survival. Pulmonary agenesis can be seen in association with congenital cardiovascular, gastrointestinal, and genitourinary malformations. Unilateral pulmonary agenesis has also been reported in association with skeletal anomalies and hydrops fetalis.
Other conditions that may result in an appearance similar to pulmonary agenesis include pulmonary aplasia and pulmonary hypoplasia. Pulmonary aplasia results in a rudimentary bronchial stump. Hypoplasia results from abnormal cessation of fetal bronchial development and may involve the entire lung or a portion of the lung.
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