Lung agenesis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Lung agenesis is defined as partial or complete failure of the lung to form with absence of the lung parenchyma, the bronchus and the pulmonary vessels.

Classification

The condition can be unilateral (compatible with life) or bilateral (fatal).

Pathophysiology

On the affected side, the pleural cavity is absent, and the healthy lung occupies the entire thorax.

Associated COnditions

• Truncus arteriosus

Epidemiology and Demographics

Lung agenesis is extremely rare.

Natural History, Complications, Prognosis

Natural History

The natural history is variable.

Complications

There can be kinking of the trachea and compression of the trachea.

Prognosis

Although 50% of patients may die before the age of 5, the remainder of the individuals may be asymptomatic throughout their lives. Bilateral lung agenesis is a lethal anomaly.