Navigating a Complex Case of Hypoplastic Right Lung With Bronchiectasis: A Ten-Year Journey.

Abstract

Unilateral pulmonary hypoplasia (UPH) is a rare congenital disorder that presents rarely in adulthood. Most patients succumb to complications at a young age, and those who survive are rare and susceptible to frequent lifelong pulmonary infections. It has a high infant mortality rate. We present the case of a 66-year-old male with rheumatoid arthritis and severe persistent asthma who first presented to our emergency department in 2013 with worsening shortness of breath. Chest imaging with a computed tomography (CT) scan revealed right hemithorax volume loss with hypoplasia, honeycomb lung formation, and right mediastinal shift. He was treated with prednisone, inhalers, and antibiotics for asthmatic bronchitis. He continued to suffer frequent hospital admissions (56 to our hospital alone) over the next decade for pneumonia and asthma exacerbations. The hypoplastic right lung was deemed to be contributing to recurrent infections/inflammation, and he is currently being re-evaluated for a right pneumonectomy, as surgical resection is an option for localized bronchiectasis associated with recurrent respiratory infections.