We report a case of unusual bronchopulmonary foregut malformation composed of a mediastinal bronchogenic cyst with sequestrated lung tissue and communicating tubular esophageal duplication associated with complete pericardial defect. A 18-yrold man, who had suffered from dry cough and mild dyspnea, was admitted because of an incidentally detected chest mass. A computed tomography scan demonstrated a cystic mass with an air fluid level connected with esophagus in the middle mediastinum. The surgically resected mass was a pleural invested accessory lobe of the lung (8.0x7.0x4.5 cm) connected with the esophageal wall by a tubular structure (3.0 cm in length and 2.0 cm in diameter). A complete left pericardial defect was also identified. Histologically, the cystic wall was composed of fibrovascular connective tissue with a smooth muscle layer, mixed seromucous glands and cartilage, and the inner surface of the cyst was lined by ciliated pseudostratified columnar epithelium. The inner surface of the tubular structure was lined by non-keratinizing or keratinizing squamous epithelium, and the wall contained submucosal mucous glands, muscularis mucosa, and duplicated muscularis propria. This case is important in understanding the embryological pathogenesis of the variable spectrum of the bronchopulmonary foregut malformation.
Adolescent ; Bronchogenic Cyst/*complications/*diagnosis ; Digestive System/pathology ; Esophageal Cyst/diagnosis/pathology ; Esophageal Diseases/*complications/*diagnosis ; Esophagus/abnormalities/*pathology ; Humans ; Lung/abnormalities/pathology ; Male ; Pericardium/pathology ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Maximal duration of intravenous (IV) corticosteroid (CS) treatment and efficacy of cyclosporin A (CsA) have not been clarified for patients with severe ulcerative colitis. We aimed to evaluate and compare the effectiveness of CS and CsA combination therapy with prolonged CS therapy alone in patients with severe UC refractory to initial CS therapy. METHODS: We retrospectively reviewed the medical records of 84 episodes of severe UC in 59 patients between April 1999 and May 2005. RESULTS: Among 84 episodes with IV CS therapy, 45 (53.6%) experienced an early response, while 39 (46.4%) did not respond within 2 weeks. The remaining 36 episodes excluding 3 which underwent colectomy were assigned to either combination therapy of IV CS and CsA or prolonged IV CS treatment alone for additional 2 weeks. Twelve of 16 episodes (75.0%) responded to therapy with combinations of IV CsA and CS, and 16 of 20 episodes (80.0%) to prolonged IV CS treatment alone. There was no statistical difference in response and colectomy rate after 4 weeks between CsA-use group and CsA-non-use group (p=1.00). CONCLUSIONS: These results suggest that CS and CsA combination has no additional benefit over prolonged CS therapy alone in terms of short-term response and that CS can be safely prolonged even after the first 14 days of treatment for severe UC.
Aged ; Aged, 80 and over ; Antibodies, Monoclonal ; Carcinoma, Squamous Cell/*metabolism/pathology ; Cyclin D1/immunology/*metabolism ; Cyclin-Dependent Kinase Inhibitor p16/immunology/*metabolism ; Esophageal Neoplasms/*metabolism/pathology ; Esophagus/*abnormalities/metabolism/pathology ; Female ; G1 Phase ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Tumor Markers, Biological/metabolism ; Tumor Suppressor Protein p53/immunology/*metabolism