1/2. Swyer-James syndrome complicated by lung abscess.Swyer-James syndrome, a rare disease with unilateral hyperlucent lung due to bronchiolitis obliterans and pulmonary artery hypoplasia, generally develops after lower respiratory tract infection during early childhood. Invasive procedures, including bronchoscopy and angiography, are often necessary for a definitive diagnosis. We report a 17-year-old man admitted because of cystic bronchiectasis complicated by lung abscess. Chest roentgenography showed the typical findings of Swyer-James syndrome. Noninvasive magnetic resonance angiography was used to confirm hypoplasia of the right pulmonary artery. The patient received antibiotic therapy, underwent a right lower lobectomy for the lung abscess, and recovered.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/2. Surgically treated Swyer-James syndrome.Because patients with Swyer-James syndrome have almost always been treated conservatively, few reports exist of pathological findings of the lung in this syndrome. We report a case of this rare disease treated surgically and discuss pathological findings. A 36-year-old woman repeatedly contracted bronchitis and pneumothorax since adolescence, until April 26, 1997, when she reported chest pain and dyspnea. Chest X-ray on admission showed left pulmonary collapse with a slight deviation of the mediastinum toward the right. Chest computed tomography showed an apical bulla and emphysematous change in the left upper lobe. Pulmonary arteriography at age 17 showed hypoplasia of left pulmonary artery branches in the left upper lobe. Based on a diagnosis of Swyer-James syndrome, we conducted left upper lobectomy on May 2, 1997. Pathological examination of the resected left upper lobe showed marked emphysematous change, including an emphysematous bulla with destruction of alveolar structure and peribronchiolar fibrosis. No vascular abnormality was recognized in histology. Emphysematous change secondary to repeated bronchiolitis is believed to have led to her repeated pneumothorax.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |