A rare non-syndromic respiratory or mediastinal malformation characterized by unilateral complete absence of lung tissue bronchi and pulmonary vessels. It may be isolated or associated with congenital malformations most commonly with heart anomalies. Presentation is highly variable including airway narrowing stridor respiratory distress recurrent respiratory tract infections and pulmonary hypertension.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version February 2024
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Syndromes Without A Name (SWAN) Australia
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