Pulmonary agenesis

Pulmonary Agenesis
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Pulmonary agenesis is a rare congenital condition characterized by the complete absence of one or both lungs, along with the absence of the bronchus and pulmonary vasculature on the affected side. This condition is a result of developmental failure during the embryonic stage.

Epidemiology

Pulmonary agenesis is an extremely rare condition, with an estimated incidence of 1 in 10,000 to 1 in 15,000 live births. It can occur as an isolated anomaly or in association with other congenital malformations.

Pathophysiology

Pulmonary agenesis occurs due to the failure of the lung bud to develop during the embryonic period. This can result in either unilateral or bilateral agenesis. Unilateral agenesis is more common and is often associated with compensatory hyperinflation of the remaining lung, as seen in the accompanying X-ray image.

Clinical Presentation

The clinical presentation of pulmonary agenesis varies depending on whether the condition is unilateral or bilateral.

• Unilateral Pulmonary Agenesis: Patients may be asymptomatic or present with respiratory distress, recurrent respiratory infections, or other respiratory complications. The mediastinum may shift towards the affected side, and the remaining lung may become hyperinflated.

• Bilateral Pulmonary Agenesis: This condition is incompatible with life, as both lungs are absent, leading to severe respiratory insufficiency immediately after birth.

Diagnosis

Diagnosis is typically made through imaging studies. A chest X-ray can reveal the absence of lung tissue on one side, mediastinal shift, and hyperinflation of the contralateral lung. Computed tomography (CT) and magnetic resonance imaging (MRI) can provide more detailed anatomical information.

Management

Management of pulmonary agenesis depends on the severity of symptoms and associated anomalies. In cases of unilateral agenesis, treatment is supportive and focuses on managing respiratory symptoms and preventing infections. Surgical interventions may be considered in some cases to correct associated anomalies or improve respiratory function.

Prognosis

The prognosis for individuals with unilateral pulmonary agenesis varies. Many individuals can lead relatively normal lives with appropriate medical management. However, the presence of other congenital anomalies can significantly affect outcomes.

Also see

• Congenital lung malformations
• Bronchopulmonary dysplasia
• Pulmonary hypoplasia