Congenital Aplasia Of Lung: A Case Report And Review Of The Literature

Objective: To describe the clinical characters together with imaging and lung function features of congenital aplasia of lung , and improve the difference-diagnosis ability to the diseases characterized by obstructive lung function.methods: One patient with congenital aplasia of lung , who had been misdiagnosed for several years , was described , and relevant literatures were reviewed.Result: Congenital aplasia of lung is relatively rare, it is classified into 3 types by Scheider, according the severity of development: Typeâ… , pulmonary agenesis, lack of one lung lobe or half a lung, even all the lung, no bronchus or blood supply, no evidence of lung substance; Typeâ…¡, pulmonary aplasia, only a blind ending of bronchi, no lung substance or blood vessels, usually happen unilaterally and have no lateral difference; Typeâ…¢, pulmonary hypoplasia, the lung appearance is almost normal, but the quantity and size of alveoli and vessels are less. 185 cases are found in the literatures, in these cases: there was no sex difference, left/right side ratio was about 2:1, typeâ… counted 9.7%, typeâ…¡23.8%, typeâ…¢66.5%; Congenital aplasia of lung were ofen misdiagnosed as bronchiectasis, pulmonary atelectasis, TB and injured lung, pleural diseased and so on; the misdiagnosis ratio was high and time was long; imagings(X-ray, CT, MRI , pulmonary angiography, and so on.), bronchoscopy, lung function test and histogram of Ventilation/Perfusion Scan, are very useful examinations to make final diagnosis. Clinical manifestations, therapy and prognosis are related to aplasia severity.Conclusion: Congenital aplasia of lung is coursed by the development disorders of the bronchi and pulmonary vessels in fetus, which is relatively rare, the etiology and pathogenesis are still unknown. most are found in newborn and children , and die; less can live for long because of slight abnormality, and diagnosed in adult or mis-diagnosed for long. The clinical manifestations are non-specific . Imagings, lung function examination, Histogram of Ventilation/Perfusion Scan, are important for the final diagnosis. Clinical manifestations, therapy and prognosis depend on the sites and severity of aplasia.