First, Etiology
It is a congenital anomaly of lung development, where the isolated lung tissue is separated from the normal lung tissue by pleura.
Second, Pathogenesis
1. Intralobar type:Common, accounting for about 75%. Most are located in the posterior basal segment of the lower lobe of the left lung, adjacent to the spine, followed by the basal segment of the lower lobe of the right lung, and less frequently in the upper lobe. Isolated lung tissue presents with varying degrees of cystic or consolidation, lacking communication with the surrounding normal tracheobronchial tree. Microscopic examination shows infiltration of monocytes and macrophages, along with fibrosis in the lung tissue, and cystic dilation of the bronchi. If the isolated lung tissue communicates with normal lung tissue, purulent secretions can be observed in the lumen, with inflammation cell infiltration in the corresponding lung tissue, such as neutrophils.
2. Extralobar type:Less common, accounting for about 25%. They are often located in the posterior basal segment of the lower lobe of the left lung, and can also be located in the mediastinum or below the intestines. The isolated lung tissue is completely separated from the normal lung tissue by pleura, and the cross-section appears brown and spongy. Microscopic examination shows underdeveloped lung tissue, even in the form of cysts. The bronchi of the isolated lung tissue do not communicate with the normal bronchi, so inflammation of the lung tissue is rare. The bronchi are often twisted and expanded to varying degrees. The blood supply of the isolated lung tissue mainly comes from the abdominal aorta or its branches, and is drained through the inferior vena cava, azygos vein, or hemiazygos vein, forming a left-right shunt. However, there are also cases where blood is supplied through the pulmonary artery and drained through the pulmonary veins.