Unilateral pulmonary hypoplasia in an adult patient

Abstract

Pulmonary hypoplasia (PH) is a developmental anomaly of the lung parenchyma, characterized by a decrease in the number and size of airways, alveoli and vessels. We present a case of a 31-year-old patient with a history of chronic productive cough and frequent respiratory infections, who was referred for investigation of abnormal chest x-ray. The combination of chest computed tomography (CT) and bronchoscopy set the diagnosis of left pulmonary hypoplasia and the patient was treated surgically with a left pneumonectomy. PH is usually diagnosed immediately after birth, causing severe respiratory failure with high mortality. The less severe, unilateral forms can possibly survive by causing compensatory hyperinflation of the other lung and remain undiagnosed until adulthood, presenting either asymptomatic or with symptoms of chronic bronchitis and recurrent respiratory infections. Chest CT is considered the imaging technique of choice for the diagnosis and for the differential diagnosis from other congenital or acquired conditions. The treatment is usually conservative, although surgical resection is indicated in cases of severe cystic changes and intense symptomatology. me Pulmonary hypoplasia (PH) constitutes a part of the spectrum of developmental anomalies that cause incomplete development of lung tissue and is characterized by a decrease in the number of lung cells, airways and alveoli, finally leading to a reduction in lung size and weight. It can be unilateral or bilateral and it usually leads to severe respiratory distress immediately after birth, while it represents a common cause of perinatal mortality [1]. The less severe forms of PH can survive and remain undiagnosed since adulthood, when they present either as an accidental imaging finding in asymptomatic patients or within investigation of recurrent lower respiratory tract infections (LRTI) and respiratory failure. Chest computed tomography (CT) is the diagnostic tool of choice [2]. A 31-year-old patient case with unilateral PH and history of frequent respiratory infections since childhood is presented.