Right upper lobe agenesis associated with dextroposition of heart and ectopic kidney in an adult patient attending pulmonary medicine outpatient department: A rare case report from South India

Abstract

Pulmonary agenesis is a rare congenital anomaly that results from the unsuccessful growth of the primitive lung buds. A case of lung agenesis was first reported in 1673 during an autopsy. Pulmonary agenesis includes agenesis, aplasia, and hypoplasia as the three most common types. The survival rate is <50% by the age of 5 years in case of agenesis. Fifty percent of the cases of lung agenesis are associated with various congenital defects such as cardiovascular, central nervous system, gastrointestinal, genitourinary, skeletal, Down syndrome, and Klippel–Feil syndrome. Here is the case of a 28-year-old male who presented with on-and-off cough and dyspnea for 3 years. On systemic examination, the patient had signs suggestive of loss of right lung volume. Chest x-ray showed right side volume loss with ipsilateral mediastinal shift with compensatory hyperinflation of the left lung. Computed tomography (CT) thorax with abdomen was suggestive of dextroposition with absent right upper lobe parenchyma, right upper lobe bronchus, right upper lobe artery, and superior pulmonary vein with the absence of hemiazygos vein with ectopic left kidney in the pelvis. Early identification of pulmonary agenesis is vital in preventing fibrosis in patients with unilateral lungs which might develop as a result of recurrent chest infections. Caution and vigilance will lead to a good prognosis.