TOPIC: Pediatrics TYPE: Fellow Case Reports INTRODUCTION: Recurrent pneumothroaces (PTX) is an uncommon presentation in the newborn period, often associated with prematurity or ventilator use. This is a case of rapidly progressive cystic disease in the setting of an absent right pulmonary artery. CASE PRESENTATION: Patient is a 18do female, ex-37 weeks gestation, with negative prenatal history. Noted at birth to have meconium aspiration at birth, cyanosis and non-invasive positive pressure ventilation (PPV) at 9 hours of life. CXR showed R PTX, that was resolved with chest tubes and discharged on day of life (DOL) 11. Presented on DOL) 17 with respiratory distress and was found to have a PTX. Chest CT showed R PTX with cystic foci in all three lobes thought to be related to pulmonary interstitial emphysema (PIE). Cardiothoracic surgery felt patient was too young for surgery and recommended more conservative management. Presented on DOL 27 with recurrrent PTX and then again at 3 months of life. Chest CT at this time showed a absent R pulmonary artery not previously recognized. ECHO confirmed absence of R PA. Patient underwent R pneumonectomy with mediastinal tent placement. Pathology showed pneumatoceles in lower lobes, focal areas of dysplastic lung development, absent large hilar pulmonary arteries, lymphangiectasis and pleural fibrosis. Cytology showed few multinucleated giant cells and fibroblastic proliferation along pneumatocele wall. DISCUSSION: Air leak syndromes such as PTX and PIE are more common in neonates with prematurity, need for PPV, meconium aspiration, and pulmonary hypoplasia. The presence of meconium aspiration and need for PPV likely contributed to the initial pulmonary insult that led to her clinical picture. Persistent PIE can associated with multiple large pseudocysts. Presence of multinucleated giant cells with fibroblast proliferation in cytology further supports presence of persistent PIE. In addition the unilateral absence of the R pulmonary artery (UAPA) can contribute to hypoperfusion and hypoplasia. This can subsequently lead to the cystic development especially when there are multiple other insults to the lung as seen in this patient. Post-pneumonectomy complications include concern for development of pulmonary hypertension as well as complication related to the post-pneumonectomy space, such as mediastinal shift causing compression of the airways. CONCLUSIONS: Recurrent PTX can be due to a wide variety of etiologies based on patient's age and presentation. This case highlights recurrent PTX in a neonate in the setting of cystic lung disease that is likely from persistent PIE with further contribution from a unilaterally absent pulmonary artery. REFERENCE #1: Jeng, M.J., Lee, Y.S., Tsao, P.C. & Soong, W.J. Neonatal air leak syndrome and the role of high-frequency ventilation in its prevention. J Chin Med Assoc 75, 551-559 (2012). REFERENCE #2: Agrons, G.A., Courtney, S.E., Stocker, J.T. & Markowitz, R.I. From the archives of the AFIP: Lung disease in premature neonates: radiologic-pathologic correlation. Radiographics 25, 1047-1073 (2005). REFERENCE #3: Kruzliak, P., Syamasundar, R.P., Novak, M., Pechanova, O. & Kovacova, G. Unilateral absence of pulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis 106, 448-454 (2013). DISCLOSURES: No relevant relationships by Ashish George, source=Web Response no disclosure on file for Robin Ortenberg; No relevant relationships by Swaroop Pinto, source=Web Response
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