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Congenital communicating bronchopulmonary foregut malformation including ectopic pancreatic tissue in an infant
Noboru Oyachi1, Fuminori Numano2, Keiichi Koizumi2
1Department of Pediatric Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Kofu, Yamanashi, 409-8506, Japan. oyachi-ampw@ych.pref.yamanashi.jp.
Insights
This study reports a rare case of bronchopulmonary foregut malformation (BPFM) in an infant. The condition involved a lung sequestration communicating with the esophagus and containing ectopic pancreatic tissue.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Congenital Malformations
Background:
- Bronchopulmonary foregut malformation (BPFM) involves communication between the lungs and gastrointestinal tract.
- BPFM associated with pancreatic tissue is rare.
- This case highlights BPFM with ectopic pancreatic tissue in an infant.
Purpose of the Study:
- To describe a rare case of bronchopulmonary foregut malformation (BPFM) in a male infant.
- To detail the pathophysiology and clinical presentation of BPFM involving pancreatic tissue.
- To contribute to the understanding of rare congenital malformations.
Main Methods:
- Diagnosis involved chest radiography and CT scans.
- Surgical resection included left lower lobectomy and sequestration resection.
- Histopathological examination confirmed the presence of pancreatic, esophageal, and gastric tissues.
Main Results:
- A male infant presented with respiratory distress and pneumonia.
- A left lower intralobar pulmonary sequestration communicating with the esophagus was identified.
- Histology confirmed ectopic pancreatic tissue within the sequestration.
Conclusions:
- A rare communicating bronchopulmonary foregut malformation (BPFM) was identified in an infant.
- The malformation included intrapulmonary sequestration, esophageal communication, and ectopic pancreatic tissue.
- This case expands the spectrum of BPFM presentations.
Background:
Several reports have documented that the pulmonary sequestration is in communication with the gastrointestinal tract and the concept of bronchopulmonary foregut malformation (BPFM) has become more widespread. However, there are few reports of the sequestration associated with the pancreas derived from the foregut. We describe the history and pathophysiology of BPFM including pancreatic tissue in a male infant with respiratory distress.
Case Presentation:
A male patient was born at 38 weeks of gestation and weighed 2752 g at birth. He developed pneumonia and was hospitalized at 3 months of age. Chest radiographs and CT scans led to the diagnosis of a lung abscess in the left lower intralobar pulmonary sequestration with aberrant arteries from the abdominal cavity. At 4 months of age, when the abscess had resolved, left lower lobectomy and the resection of the intralobar sequestration were performed. The pulmonary sequestration was conjoined with the esophagus. A fistula was found between the lower esophageal wall and the pulmonary sequestration. An additional small segment of the esophageal wall was excised. Histologically, the mediastinal surface of the sequestration tissue contained pancreatic tissue. Furthermore, esophageal and gastric tissue, cartilage tissue, and ciliated epithelium were confirmed. A definitive diagnosis of BPFM was made.
Conclusions:
We postulated the rare case of a communicating BPFM with intrapulmonary sequestration on one end and the esophagus on the other forming a mass lesion, which included ectopic pancreatic tissue in a male infant.
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