SESSION TYPE: Miscellaneous Case Report Posters II
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Complete unilateral pulmonary agenesis is an extremely rare congenital condition, characterized by complete absence of bronchi, parenchyma, vessels and pleural cavity in the affected lung. Fifty percent of infants born with pulmonary aplasia are stillborn or die within the first five years of life. We present a patient with right pulmonary agenesis complicated by ASD that has survived to adulthood.
CASE PRESENTATION: A 21 year-old female presents with new onset of hypoxic respiratory failure and dyspnea after diagnosis of community-acquired pneumonia. She has a right pulmonary agenesis, dextrocardia and ASD. Initial diagnosis in the first week of life and ASD surgical repair at 7 months of age, complicated with chronic hypoxic respiratory failure and tracheostomy till age of 5. She also has severe pulmonary hypertension. She was lost to follow up until the current presentation. She was not on supplemental oxygen until current hospitalization. Community-acquired pneumonia treated with Moxifloxacin 10 days course, discharged on 6 L/min of oxygen at rest. Physical exam: BMI 45, vesicular breath sounds over left chest and transmitted breath sounds over right chest, normal S1 and loud S2 over right hemithorax. Echocardiogram: dextrocardia, estimated RV pressure of 89 mmHg with moderate mitral regurgitation. PFT shows obstruction with FEV1/FVC 68%, FEV1 0.81 L (28.7%) and FVC 1.2 L (37.9 %). Chest roenterogram: homogenously opaque right hemithorax with mediastinal shift and hyperlucency of left lung field. CT chest: left lung emphysema, right mediastinal shift, right lung agenesis and heart in right hemithorax.
DISCUSSION: There is no specific therapy for pulmonary agenesis. We took a symptomatic approach to therapy, treating her obstructive lung disease with Albuterol, Tiotropium, and Mometasone, pulmonary rehabilitation with improved functional status. Planned left and right cardiac catheterization to evaluate for any remediable anatomic cardiovascular problems that may not be evident by ECHO but may contribute to pulmonary hypertension and assess responsiveness to pulmonary vasodilators.
CONCLUSIONS: Lung aplasia is often associated with acute respiratory distress and a high mortality rate early in life. This case highlights the fact that children with complicated congenital pulmonary diseases are increasingly living into adulthood. As adult pulmonologists, we must be more aware of these diseases and their pathophysiological changes to be able to adequately diagnose and treat our patients.
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DISCLOSURE: The following authors have nothing to disclose: Olena Lineberry, P.Jim Murphy, Craig Piquette, Kristina Bailey
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