SESSION TITLE: Pediatrics and Congenital Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Tuesday, October 27, 2015 at 07:30 AM - 08:30 AM
INTRODUCTION: In young patients, the most common causes of dyspnea include asthma, deconditioning, and psychosomatic manifestations of anxiety. Less common are ILD, cardiovascular disease, and pulmonary vascular disease. We present a case of woman with shortness of breath due to asthma and agenesis of her right lung.
CASE PRESENTATION: A 27-year-old woman presented with dyspnea on exertion. She reported three URIs in the past year requiring antibiotics. She was diagnosed with dextrocardia as a young child but denied frequent childhood infections. There was no personal or family history of infertility. She had clear lungs, normal heart sounds, and displaced PMI to the right mid-clavicular line. Her echocardiogram was normal and PFTs showed severe obstruction, FEV1 1.5L (43% predicted), a robust bronchodilator response, mild air trapping, and preserved DLCO. A chest x-ray showed dextrocardia, right-sided volume loss, and flattened diaphragms. A chest CT revealed right-sided mediastinal shift with cardiac dextroposition, a single pulmonary trunk continuing as the left main pulmonary artery, a single left mainstem bronchus, and extensive compensatory hyperinflation of the left lower and upper lobe into the right thorax. Her asthma was treated with inhaled glucocorticoids with moderate clinical improvement.
DISCUSSION: Lung agenesis is a rare congenital disorder. Varying degrees of agenesis exist, including hypoplasia with decreased volume of normal parenchyma and vasculature, aplasia with a rudimentary bronchus and central vasculature but absent lung parenchyma, and complete agenesis of one lung and its associated vasculature. Patients have repeated infections which may result in bronchiectasis. 50% of patients have other congenital defects. Our patient has complete lung agenesis, infrequent infectious complications, and no other congenital defects or organ malposition. Her remaining lung has completely expanded to fill the entire thoracic cavity and flatten her diaphragms, which is often not the case in single lung agenesis. We believe the dyspnea to be a product of reversible airway obstruction and compensatory lung hyperinflation.
CONCLUSIONS: Shortness of breath is a common presenting complaint. While asthma was diagnosed and treated, the history of repeated respiratory infections and dextrocardia should prompt a thorough evaluation into underlying congenital lung disease.
Reference #1: M.K. Mardini, W.L. Nyhan. Agenesis of the lung:Report of four patients with unusual anomalies. Chest, 87 (1985), pp. 522-527
DISCLOSURE: The following authors have nothing to disclose: Michael Bergman, Navitha Ramesh, Sarun Thomas, Jason Filopei, Alfred Astua
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