J Korean Med Sci.  2007 Jun;22(3):564-567. 10.3346/jkms.2007.22.3.564.

Unusual Bronchopulmonary Foregut Malformation Associated with Pericardial Defect: Bronchogenic Cyst Communicating with Tubular Esophageal Duplication

• Eom DW
• Kang GH
• Kim JW
• Ryu DS

• KMID: 1778373
• DOI: http://doi.org/10.3346/jkms.2007.22.3.564

Abstract

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.

Keyword

Pulmonary Sequestration; Bronchogenic Cyst; Pericardium; Defect

MeSH Terms

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

Figure

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  Fig. 1 Axial chest CT on lung setting shows a cyst (short arrow) with an air-fluid level (long arrow) abutting the esophagus by tubular structure (arrow head) in the aortopulmonary window.

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  Fig. 2 Axial contrast enhanced CT shows an abnormal interposition of the lung tissue (arrowhead) between the aorta and the main segment of the pulmonary artery, indicating the absence of the pericardium.

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  Fig. 3 The cut surface of the mass shows a unilouclar cyst containing soap bubble-like material, which is connected with tubular structure. The investing lung parenchyma shows a subpleural bullous formation.

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  Fig. 4 Microscopic features of the specimen (H&E ×40). (A) The wall of tubular structure lined by squamous epithelium contains duplicated muscular layer and submucosal mucous glands. (B) The wall of bonchogenic cyst lined by ciliated pseudostratified columnar epithelium is composed of fibroconnective tissue, hyaline cartilage, and mucous glands. (C) The investing lung parenchyma shows of mature alveolar spaces with an emphysematous change. Some alveolar spaces are filled with proteinaceous fluid. (D) A transition from squamous epithelium to columnar epithelium is noted in the junction of the bronchogenic cyst and tubular esophageal duplication.

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