Chest Imaging

Bronchopulmonary sequestration

nonfunctioning lung tissue, usually not in continuity with the tracheobronchial tree, deriving its blood supply from the systemic circulation. The pulmonary sequestration spectrum encompasses a large group of diseases embryologically related to developmental anomalies of lung, bronchi, pleura, pulmonary and systemic vessels, diaphragm and the foregut derivatives. It is usually classified into two groups: intralobar and extralobar. Intralobar sequestration accounts for 75%. Its pleuroparenchymal component includes consolidation, mass or cyst(s) within a lower lobe. The pleural cavity is common to the normal lung and to the malformation (Fig.1). The systemic supply originates from the lower part of the thoracic aorta or from the upper abdominal aorta. It can be the only anomaly without pleuroparenchymal malformation. The venous drainage is pulmonary. Extralobar sequestration lies outside the normal investment of the visceral pleura and drains into the azygoshemiazygos system or the portal vein. It can be supra-, intra- or subdiaphragmatic. While extralobar sequestration is a congenital anomaly often discovered in neonates or infants, intralobar sequestration is often discovered in adults as a solitary defect and may be an acquired consequence of chronic obstruction to a bronchus by a foreign body or an endobronchial tumour.

Whether intralobar or extralobar the bronchopulmonary component of these anomalies has to be surgically treated. Preoperative management requires topography of the lung anomaly and of its vascular connections. It can be noninvasively obtained with ultrasonography, CT or MRI (Fig.2). Anomalous systemic arterial supply to normal lung can be treated with lobectomy, anastomose of the anomalous artery to the pulmonary artery, ligation or embolization of the systemic supply. Angiography is only required before embolotherapy. The intralobar purely vascular sequestration requires invasive treatment, even when asymptomatic, due to the risks of vascular complications such as left to left shunting, cardiac failure and haemoptysis. Also, see sequestration.

JR - MRJ

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Fig.1

Bronchopulmonary sequestration corresponding to a purely vascular intralobar sequestration. The anomaly is responsible for massive bleeding of the left lower lobe.
Bronchopulmonary sequestration, Fig.1
Bronchopulmonary sequestration, Fig.2 (a)
Bronchopulmonary sequestration, Fig.2 (b)