NOT JUST FOR THE KIDS: A CASE OF PULMONARY HYPOPLASIA DIAGNOSED IN ADULTHOOD

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Pulmonary hypoplasia is a developmental abnormality characterized by variable maldevelopment of the bronchial tree, pulmonary parenchyma, and supporting structures. It is a clinically relevant cause of respiratory failure and mortality in newborns and infants but a rare diagnosis in adulthood. CASE PRESENTATION: A 49-year old woman with a past medical history significant for pulmonary tuberculosis (TB) presented to the Emergency Department (ED), sent in for an abnormal chest x-ray (CXR). The patient had seen her primary care physician for sinus congestion, malaise, dyspnea and cough. She had an uncomplicated birth, no frequent childhood illnesses, but did have frequent episodes of bronchitis as an adult. She had been treated for non-cavitary TB 15 years ago with a four-drug regimen. An outpatient CXR revealed a near complete opacification of the left hemithorax, mediastinal shift to the left, and a hyperinflated right lung. There were absent breath sounds in the left lung except for at the left apex. CT scan showed severe hypoplasia of the left lung, a thickened but narrowed left mainstem bronchus, and opacification of the left lower lobe bronchi. Pulmonary function tests were normal. Fiberoptic bronchoscopy revealed severe narrowing of the left mainstem bronchus with a hypoplastic left upper and lower lobe bronchus too narrow for direct visualization. Administration of saline was highly suggestive of early termination of both bronchi. Cytology, pathology, cultures, including Acid Fast Bacilli smears, were negative. The patient was ultimately diagnosed with unilateral pulmonary hypoplasia and discharged with close outpatient follow up. DISCUSSION: Pulmonary hypoplasia is an important cause of respiratory failure in neonates and children associated with other fetal abnormalities, but some adults remain asymptomatic due to compensatory hypertrophy of the contralateral lung. Depending on the degree of lung development, a chest x-ray will often show a completely opacified hemithorax, an ipsilateral shift of the mediastinum, and hyperinflation of the contralateral lung. For a definitive diagnosis, CT scan is the preferred modality; however, in some instances, bronchoscopy may be helpful to rule out sizeable endobronchial lesions with severe atelectasis, which may mimic pulmonary hypoplasia. These patients are at a higher risk of developing pneumothoraces, pulmonary hypertension, and frequent, more serious respiratory infections due to underdeveloped alveolar tissue and decreased mucociliary clearance and surfactant. CONCLUSIONS: Pulmonary hypoplasia should be on the differential for any patients presenting with opacification of hemithorax on CXR. A CT scan, a ventilation-perfusion scan, and bronchoscopy are modalities often used to establish the diagnosis. Reference #1: Katsenos, S., Antonogiannaki, E., & Tsintiris, K. (2013). Unilateral Primary Lung Hypoplasia Diagnosed in Adulthood. Respiratory Care, 5 Reference #2: Georgescu, A., Nuta, C., & Bondari, S. (2011). 3D Imaging in Unilateral Primary Pulmonary Hypoplasia in an Adult: A Case Report. Case Reports in Radiology, 2011, 1-6. https://doi.org/10.1155/2011/659586 Reference #3: Kurkcuoglu, I. C., Eroglu, A., Karaoglanoglu, N., & Polat, P. (2005). Pulmonary Hypoplasia in a 52-Year-Old Woman. The Annals of Thoracic Surgery, 79(2), 689-691. https://doi.org/10.1016/j.athoracsur.2003.09.122 DISCLOSURES: stock relationship with Pfizer Please note: $1001 - $5000 Added 03/11/2019 by Zubair Hasan, source=Web Response, value=stock stock relationship with Stryker Please note: $1001 - $5000 Added 03/11/2019 by Zubair Hasan, source=Web Response, value=stock stock relationship with United Health Group Please note: $1001 - $5000 Added 03/11/2019 by Zubair Hasan, source=Web Response, value=stock No relevant relationships by Sameer Khanijo, source=Web Response No relevant relationships by Dae Hyeon Kim, source=Web Response