Congenital Abnormalities of Intrathoracic Airways
Section snippets
Embryology
Normal tracheobronchial development is initiated at 24 to 26 days as a median bulge of the ventral wall of the pharynx which develops at the caudal end of the laryngotracheal groove. At 26 to 28 days, it gives rise to right and left lung buds. As the lung buds elongate, the trachea is separated from the esophagus by lateral ingrowth of the mesoderm forming the tracheoesophageal septum. The endodermal lining of the laryngotracheal tube gives rise to the epithelium of the tracheobronchial tree,
Developmental interruption
Congenital underdevelopment of the tracheobronchial tree and the lungs, known as the agenesis-hypoplasia complex, has been classified into three groups according to the embryologic stage of developmental interruption: (1) agenesis (absence of bronchus, vessel, and lung parenchyma), (2) aplasia (absence of lung parenchyma with blind rudimentary bronchus present), and (3) hypoplasia (decrease of the number and size of bronchi, vessels, and parenchymal structures).19 Diagnosis is easily
Communicating bronchopulmonary foregut malformations—esophageal bronchus
Communicating bronchopulmonary foregut malformations (CBPFMs) are uncommon anomalies characterized by a patent congenital communication between a portion of respiratory tract on one side and the esophagus or stomach on the other side.27, 28, 29, 30, 31, 32 Concomitant congenital tracheobronchial stenoses have been reported.33, 34 CBPFMs can also occur in combination with other congenital anomalies involving the pulmonary and systemic vascular systems, diaphragm, upper gastrointestinal tract,
Tracheal Atresia
Tracheal atresia is an exceptional anomaly in which a segment of the trachea, generally proximal, is missing.9 The anomaly is lethal except when associated with a tracheo-esophageal fistula, allowing esophageal intubation and subsequent surgical reconstruction.9, 44 Three types of tracheal atresia have been described depending on the tracheobronchial segment that communicates with esophagus, with the carina being the most frequent (60%) site of communication.9, 20, 45 Prenatal diagnosis of such
Ectopic or supernumerary lung buds
Noncommunicating bronchopulmonary foregut malformations, including among others bronchogenic cysts, result from abnormal budding of the developing tracheobronchial tree. Pulmonary sequestration, CCAM, and congenital lobar emphysema are diseases of lung tissue.23, 25, 26 Those anomalies are beyond the scope of this article and are not discussed further herein.
Malformations associated with abnormalities of situs
Situs solitus is defined as the modal arrangement of organs and vessels within the body. The right-sided lung is trilobed, and the right atrium is the systemic one receiving blood from the vena cava. On the left side, the lung is bilobed, and the left atrium is the pulmonary one receiving blood from the pulmonary veins. Situs anomalies imply a disordered organ arrangement in the chest and/or abdomen.
Summary
Recognition of bronchial variants and anomalies by the radiologist is an important step in reporting on CT examinations of the chest because it has numerous clinical implications. Precise description is necessary for the pneumologist (for fiberoptic bronchoscopy, bronchoalveolar lavage, biopsy, and endobronchial treatment), the chest surgeon (for lung resection and transplantation), and the anesthesiologist (for endotracheal tube placement).
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