| خلاصه مقاله | Congenital lung disease
CPAM: hamartomatous malformation of terminal bronchioles that maintain communication with bronchial tree. Base of cyst size, it has three type. Its prognosis is mainly dependent on size of lesion rather than its type.
Bronchial atresia: a round or oval central lung opacity with adjacent lung parenchymal hyperlucency. Most common in left upper lobe, followed by right upper and right middle lobes. Most patients are asymptomatic.
Neonatal lobar hyperinflation: previously known as congenital lobar emphysema, caused by bronchial narrowing with resultant air trapping, but without associated destruction of lung parenchyma. Respiratory difficulties are usually evident within the first month of life.
Radiographically, hyperlucency of the affected lobe is seen with compression of adjacent lung diaphragmatic depression, and contralateral mediastinal shift. Treatment is surgical for symptomatic patients, whereas relatively asymptomatic patients are observed for spontaneous resolution.
Bronchopulmonary sequestration: consists of nonfunctioning lung tissue lacking normal communication with the tracheobronchial tree.
Usually receive arterial blood supply from the thoracic aorta. Divided into intralobar and extralobar forms. Former one by ratio of 3 to 1 is more common.
A major differentiating feature between two types is the arterial supply to and venous drainage from the sequestered lung.
Hypogenetic lung-scimitar syndrome: characterized by an underdeveloped right lung with abnormal venous drainage of the lung inferior vena cava just above or below the right hemidiaphragm. The systemic venous drainage of the lung produce an extracardiac left to right shunt. The classic appearance of a solitary scimitar vein is seen only one-third of cases. Most patients are asymptomatic, some may present with recurrent infection or symptoms related to a left-to-right shunt or the associated cardiac anomalies. |