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Congenital bronchopulmonary foregut malformations. Intralobar and extralobar pulmonary sequestrations communicating
R H Hruban1, S J Shumway, S B Orel
1Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Insights
Two rare cases of bronchopulmonary foregut malformations highlight their diverse presentations. These findings suggest intralobar and extralobar pulmonary sequestrations with foregut communication are related thoracic disorders.
Area of Science:
- Thoracic Surgery
- Pediatric Surgery
- Congenital Malformations
Background:
- Bronchopulmonary foregut malformations (BPFMs) encompass a spectrum of congenital thoracic anomalies.
- Pulmonary sequestrations, both intralobar and extralobar, can present with foregut communication, though typically associated with extralobar types.
Observation:
- A 12-year-old male with pectus excavatum developed lobar emphysema secondary to an intralobar pulmonary sequestration with esophageal communication.
- A 27-year-old female presented with an extralobar pulmonary sequestration in the anterior mediastinum, primarily supplied by the pulmonary artery, also communicating with the esophagus.
Findings:
- These cases demonstrate unusual variants of BPFMs, challenging previous associations.
- The intralobar sequestration with foregut communication in a pediatric patient and the anterior mediastinal extralobar sequestration with atypical vascular supply are noteworthy.
Implications:
- The presented mixed forms support a unified hypothesis linking various pulmonary sequestrations and foregut communications as related thoracic disorders.
- Understanding these variations is crucial for accurate diagnosis and management of BPFMs.
Abstract:
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.