Congenital bronchopulmonary foregut malformations. Intralobar and extralobar pulmonary sequestrations communicating with the foregut. 1989

R H Hruban, and S J Shumway, and S B Orel, and J S Dumler, and R R Baker, and G M Hutchins
Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland.

Two unusual variants of bronchopulmonary foregut malformations are presented. The first case was that of a 12-year-old male with a history of pectus excavatum in whom severe lobar emphysema developed secondary to an intralobar pulmonary sequestration that communicated with the esophagus. This case was unusual in that foregut communications and associated congenital anomalies are generally believed to be restricted to extralobar pulmonary sequestrations. The second case was that of a 27-year-old woman with an extralobar pulmonary sequestration that communicated with the esophagus. The sequestration was unusual in that it arose in the anterior mediastinum and received the bulk of its blood supply from the pulmonary artery. The occurrence of mixed forms of pulmonary sequestrations supports the hypothesis that extralobar and intralobar sequestrations and sequestrations with foregut communication are related thoracic disorders that are best considered bronchopulmonary foregut malformations.

UI MeSH Term Description Entries
D008168 Lung Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood. Lungs
D008297 Male Males
D008479 Mediastinal Neoplasms Tumors or cancer of the MEDIASTINUM. Cancer of Mediastinum,Mediastinal Cancer,Cancer of the Mediastinum,Mediastinum Cancer,Mediastinum Neoplasms,Neoplasms, Mediastinal,Cancer, Mediastinal,Cancer, Mediastinum,Cancers, Mediastinal,Cancers, Mediastinum,Mediastinal Cancers,Mediastinal Neoplasm,Mediastinum Cancers,Mediastinum Neoplasm,Neoplasm, Mediastinal,Neoplasm, Mediastinum,Neoplasms, Mediastinum
D001998 Bronchopulmonary Sequestration A developmental anomaly in which a mass of nonfunctioning lung tissue lacks normal connection with the tracheobroncheal tree and receives an anomalous blood supply originating from the descending thoracic or abdominal aorta. The mass may be extralobar, i.e., completely separated from normally connected lung, or intralobar, i.e., partly surrounded by normal lung. Pulmonary Sequestration,Bronchopulmonary Sequestrations,Pulmonary Sequestrations,Sequestration, Bronchopulmonary,Sequestration, Pulmonary,Sequestrations, Bronchopulmonary,Sequestrations, Pulmonary
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D004646 Emphysema A pathological accumulation of air in tissues or organs.
D004947 Esophagus The muscular membranous segment between the PHARYNX and the STOMACH in the UPPER GASTROINTESTINAL TRACT.
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000015 Abnormalities, Multiple Congenital abnormalities that affect more than one organ or body structure. Multiple Abnormalities

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