SESSION TITLE: Global Case Report Posters SESSION TYPE: Global Case Reports PRESENTED ON: October 18-21, 2020 INTRODUCTION: Introduction Congenital bronchopulmonary vascular malformations include a wide spectrum of disorders that compromise one or more of the three main systems of the lung (lung parenchyma, arteries and veins). The stage of gestation where disruption of the pulmonary vessels occurs determines the severity of lung affectation and alveolarization, ranging for segmentary affectation or the complete pulmonary hypoplasia. We present a case of primary pulmonary hypoplasia secondary to pulmonary artery stenosis. CASE PRESENTATION: A 11-year-old boy with history of recurrent pneumonia is evaluated in clinic. He was first admitted to the intensive care unit at age 40 months for pneumonia. Since then, he has had multiple admissions for recurrent pulmonary infections and wheezing treated with beta agonists. His chest x-ray revealed a marked decrease in volume in the right lung, with ipsilateral mediastinal shift. Computerized tomography angiography showed generalized bronchiectasis, main bronchus and pulmonary artery hypoplasia in the right side, with a significant reduction in lung volume. The left lung was hyperinflated and had compensatory emphysema. Scintigraphy revealed a total absence of perfusion in the right lung. Right heart catheterism was consistent with pulmonary artery stenosis. Patient underwent right pneumonectomy. He developed pneumonia and hydrothorax in the postoperative period, and was treated conservatively with antibiotics and incentive spirometer use. Repeat imaging showed a decrease in pleural effusion size. He had clinical improvement and he was discharged home with ambulatory follow up. DISCUSSION: Pulmonary hypoplasia is frequently associated with other malformation, including abnormalities of the thoracic cavity, masses and amniotic fluid disorders. When severe, it is generally diagnosed prenatally or shortly after birth. It can also be associated with congenital heart defects and in rare cases, no cause can be found. The severity of symptoms and their time of occurrence is defined by the degree of hypoplasia present in the patient. There have been reports of diagnosis in adulthood, presenting with recurrent respiratory infections. Clinical exam can show a shorter hemitorax of the affected side, intercostal spaces or diminished breath sounds. Imaging studies like CT can aid diagnosis, showing deviation of the mediastinum to the affected side, thinner vasculature and enlarg. CONCLUSIONS: Pulmonary hypoplasia secondary to pulmonary artery stenosis might present as recurrent respiratory infections and hospitalization. In cases with severe hypoplasia where lung function is severely compromised, pneumonectomy can be considered. Reference #1: Katayama Y, Kusagawa H, Komada T, Shomura S, Tenpaku H. Bronchopulmonary foregut malformation. Gen Thorac Cardiovasc Surg. 2011;59(11):767-770. doi:10.1007/s11748-010-0763-z Reference #2: Abrams, M., Ackerman, V. & Engle, W. Primary Unilateral Pulmonary Hypoplasia: Neonate through Early Childhood — Case Report, Radiographic Diagnosis and Review of the Literature. J Perinatol 24, 667–670 (2004). https://doi.org/10.1038/sj.jp.7211156 Reference #3: Papadopoulos D, Misthos P, Chorti M, et al. Unilateral pulmonary hypoplasia in an adult patient. Monaldi Arch Chest Dis. 2018;88(1):829. Published 2018 Jan 29. doi:10.4081/monaldi.2018.829 DISCLOSURES: No relevant relationships by Amarilys Alarcon-Calderon, source=Web Response No relevant relationships by ISMAR LOPEZ MURALLES, source=Web Response No relevant relationships by LUIS PEREZ MANCILLA, source=Web Response No relevant relationships by Shubert Iván Quiñónez Najera, source=Web Response
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