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[Agenesis of the lung].

Agenesis of the lung is a rare anomaly of unknown etiology. Recently, a 25-year old female with no known genetic disposition or teratogenic exposition was delivered of a female child in the 37th week of a normal pregnancy. The delivery was uncomplicated and normal. Shortly after birth the child developed respiratory distress. A chest X-ray showed displacement of the heart towards the left and a dense opacity of the left hemithorax (Fig. 1). Congenital atelectasis was suspected and antibiotic treatment with ampicillin and gentamycin initiated. As the child's condition did not improve, respirator treatment became necessary, Three days after birth cineangiocardiography revealed a threadlike remnant of the left pulmonary artery with no recognizable peripheral branches. The heart and mediastinum were displaced towards the left. The ductus arteriosus was patent and no cardiac anomalies were found. The second left rib was hypoplastic: Furthermore, the child had a hypoplastic left hemifacies with two preauricular tags. The chromosome content of cells from these and of circulating lymphocytes was normal, 46 XX. In neonates agenesis of the lung and congenital atelectasis should be considered when finding a dense opacity on one side and a large translucent lung on the other [1]. The diagnosis of agenesis is confirmed by cineangiocardiography when pulmonary perfusion is absent on one side [2]. Our patient had a hypoplastic rib an anomaly which is extremely rarely reported in combination with pulmonary agenesis [3]. However, this condition can be associated with malformations of almost any organ or tissue (cardiovascular, neuro-musculo-skeletal, and visceral). The prognosis is to some extent determined by the associated anomalies and almost 50% die in the first year of life [4].