We report here on a case of a rare, complex bronchopulmonary foregut malformation (BPFM) that was composed of an extralobar pulmonary sequestration communicating with an esophageal duplication cyst. A 33-year-old female presented with an incidentally detected chest mass. The computed tomography revealed a 7.5 x 4.0 cm sized heterogeneous, solid and cystic lesion in the right superior mediastinum.
Surgical resection demonstrated the solid portion to be isolated lung tissue invested in its own pleura. A unilocular cyst was communicating with the bronchus of the sequestrated lung, and microscopically the cyst was lined by squamous epithelium overlying the thick layers of smooth muscle. This case is important for understanding the spectrum of BPFMs and for differentiating a mediastinal mass, especially one at the unusual location.
Citations
Citations to this article as recorded by
Prenatal ultrasound, magnetic resonance imaging and therapeutic options for fetal thoracic anomalies: a pictorial essay Pablo Caro-Domínguez, Teresa Victoria, Pierluigi Ciet, Estrella de la Torre, Ángel Chimenea Toscano, Lutgardo García Diaz, José Antonio Sainz-Bueno Pediatric Radiology.2023; 53(10): 2106. CrossRef
Concurrent bronchopulmonary foregut malformations: a rare case of right-sided extralobar pulmonary sequestration and bronchogenic cyst Carolyn Hanna, Priya G. Sharma, Moiz M. Mustafa, Jennifer Reppucci, Archana Shenoy, Dhanashree Rajderkar Egyptian Journal of Radiology and Nuclear Medicine.2021;[Epub] CrossRef
Because early embryonic development of the tracheobronchial tree and foregut are closely associated, there is a wide spectrum of congenital anomalies involving either one or both organ systems. We analysed a total of 89 surgical and autopsy cases that are assumed to belong to congenital bronchopulmonary foregut malformation from the files of Seoul National University Hospital and Children's Hospital during the periord of 1961~1990. We also reviewed the serial sections of the embryos and fetuses from 3 weeks to fifteen weeks fertilization age for the observation of tracheobronchial and esophageal trees. Intralobar sequestrations(25 cases) and extralobar pulmonary sequestrations(4 cases) with patent, involuted-partial or complete-communication with the alimentary tract, tracheoesophageal fistula(30 cases) with or without esophageal atresia, esophageal atresia, esophageal stenosis due to tracheobroncheal remnant(4 cases), foregut duplication cysts(3 cases), esophageal or gastric diverticulum(1 cases), and bronchogenic cysts(22 cases) are included in this analysis(Table 1). Through this study, we confirmed the unifying concept of "bronchopulmonary forgut malformations". We believe a common embryologic pathogenesis leads to the formation of a previously described spectrum of malformations.