SESSION TYPE: Miscellaneous Student/Resident Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Swyer-James Syndrome (SJS), also known as idiopathic unilateral hyperlucent lung, is a rare condition arising in infancy or early childhood. It is considered a post-infectious form of bronchiolitis obliterans, caused by infectious agents such as Adenovirus, Parainfluenza virus, Respiratory Syncytial Virus, Influenza virus, and less commonly, Mycoplasma pneumoniae. SJS is characterized by a diminished number and size of pulmonary vessels, and overdistention of alveoli, resulting in a unilateral hyperlucent lung by radiography.
CASE PRESENTATION: A 14 year-old Caucasian girl presented with a chronic dry cough and dyspnea on exertion. Two weeks earlier, she developed respiratory difficulty, a chest radiograph and CT scan revealed cavitary pneumonia in the left lower lobe and an area of hyperlucency bilaterally. Although typically seen unilaterally, the imaging remained otherwise consistent with a diagnosis of SJS. Her past history included pneumonia due to Mycoplasma pneumoniae at six years of age. Physical exam revealed decreased breath sounds in the left upper posterior lung field with no adventitious breath sounds. Pulmonary function testing revealed obstructive lung disease. She was treated with steroids and bronchodilators, with improvement in her symptoms.
DISCUSSION: Most patients with SJS are asymptomatic. It is typically discovered as an incidental finding on chest radiograph. When symptomatic, it can mimic asthma or other allergic processes. Additional complications include pneumonia, as observed in this patient, lung abscess, and spontaneous pneumothorax. The differential diagnosis of unilateral pulmonary hyperlucency includes post-inflammatory stricture, congenital anomalies, tumor, atelectasis, foreign body, and pulmonary agenesis. A history of a severe respiratory infection as an infant or child, as described in this case, is common in patients diagnosed with SJS. Standard management, as with this patient, includes symptomatic control with steroids and bronchodilators, and antibiotics for recurrent pneumonia.
CONCLUSIONS: This case illustrates a number of key features of SJS including a prior history of a severe pulmonary infection in childhood, and a current presentation with pneumonia prompting imaging of the chest, which led to our diagnosis. Most patients with SJS achieve symptomatic control and live a normal life.
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2) Moonnumakal SP, Fan LL: Bronchiolitis obliterans in children. Curr Opin Pediatr 2008; 20:272-278.
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DISCLOSURE: The following authors have nothing to disclose: Dustin Snapper, Kevin Maupin, Safina Kureshi
No Product/Research Disclosure InformationWest Virginia University School of Medicine, Charleston, WV