Contrast enhanced computed tomogram showed absence of left lung and herniation of right lung to the left and bronchiectatic changes in right lung(Fig. 3),absence of left main bronchus and left pulmonary artery(Fig. 2).Fibreoptic bronchoscopy showed only a dimple at the place of the opening of left main bronchus (Fig. 4). Ultrasonogram revealed pericardial effusion. He was diagnosed as left sided agenesis of lung with left pulmonary artery atresia, pulmonary
artery hypertension and bronchiectasis of right lung. Unilateral agenesis of the lung may be present to varying degrees of severity. The left lung is affected more frequently than the right, males predominate over females and the majority of cases exhibit other congenital abnormalities like patent ductus arteriosus, pulmonary artery atresia, cardiac malformation, tracheo-esophageal fistula, cardiac malformation and horse-shoe kidney.However,several STAT inhibitor older reports prove that other anomalies are more associated with right sided agenesis and persons with right sided agenesis mostly die within first year of their life,due to associated cardiac malformations.2 Originally Schneider (1912) 3 classified agenesis into three groups which was later on modified by Boyden 4 as- click here Type I(Agenesis): Complete absence of lung and bronchus and absence of blood vessels to the affected side. Type II(Aplasia):Rudimentary
bronchus with complete absence of lung tissue. Type III(Hypoplasia):Presence of variable amounts of lung parenchyma,bronchial tree and supporting vasculature. Our patient has been classified as Type I. In Schneider’s agenesis 4-Aminobutyrate aminotransferase grade I and II, the affected side contains no lung tissue, and only the existing lung gets the branch from the main pulmonary artery, an observation which has been confirmed several times and has been seen in our case also. Clinical presentation of agenesis lung is marked by its variety from recurrent childhood respiratory infection resulting from imperfect drainage of lung secretions or from the spillover of pooled secretions
from a blind bronchial stump into initially normal lung tissue, frequent haemoptysis due to bronchiectasis of remaining lung to major organ malformation leading the patient to succumb in early life. A similar case was reported in Turkey as, a 30-year-old man presenting with dyspnoea was diagnosed to have right lung agenesis and left pulmonary bronchiectasis.5 Autosomal recessive chromosomal aberration,associated with consanguineous marriage6,deficiency of vitamin A,intrauterine infections, environmental factors have been held responsible for the etiology of congenital lung malformations. During normal development, the heart shifts to the left in the 4th week of foetal life and simultaneously the trachea develops as a ventral diverticulum arising from the foregut.7 Pulmonary agenesis or aplasia occurs perhaps due to the failure of the bronchial analogue to divide equally between the two lung buds.