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Communicating bronchopulmonary foregut malformations: classification and embryogenesis
M S Srikanth1, E G Ford, P Stanley
1Division of Pediatric Surgery, Childrens Hospital of Los Angeles, CA.
Insights
Communicating bronchopulmonary foregut malformations (CBPFMs) are rare congenital anomalies. This study proposes a new classification and embryogenesis theory for CBPFMs, aiding diagnosis in infants with respiratory issues.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Congenital Malformations
Background:
- Communicating bronchopulmonary foregut malformations (CBPFMs) involve abnormal connections between respiratory tissue and the foregut.
- These rare congenital anomalies present diagnostic challenges in neonates and infants.
Observation:
- A review of 6 cases (1959-1989) and 51 reported patients was conducted.
- A novel classification system for CBPFMs was developed based on anatomical and embryological features.
Findings:
- Group I (16%): Associated with esophageal atresia and tracheoesophageal fistula.
- Group II (33%): Lung originates from the lower esophagus.
- Group III (46%): Isolated lung lobe/segment communicates with esophagus/stomach.
- Group IV (5%): Bronchial system communicates with the esophagus, receiving systemic blood supply.
Implications:
- The proposed embryogenesis theory suggests CBPFMs arise from abnormal lung bud development during foregut partitioning.
- CBPFMs should be considered in infants with respiratory distress or recurrent pneumonias.
- Contrast studies are crucial to differentiate CBPFMs from pulmonary sequestration.
Abstract:
Communicating bronchopulmonary foregut malformations (CBPFMs) are characterized by a fistula between an isolated portion of respiratory tissue (ie, a lung, a lung lobe, or a segment) and esophagus or stomach. We combine our 30-year (1959 to 1989) experience of 6 cases with 51 reported patients to propose a CBPFM classification supported by a proposed embryogenesis theory. Group I (16%): anomaly is associated with esophageal atresia and tracheoesophageal fistula. Group II (33%): one lung originates from the lower esophagus. Group III (46%): an isolated anatomic lung lobe or segment communicates with the esophagus or stomach. Group IV (5%): A portion of the normal bronchial system communicates with the esophagus. The portion of the lung served by the communicating bronchus receives systemic blood supply. The right and left lung sacs curve dorsally to embrace the lower esophagus during normal lung development. At this stage a part of the lung bud joins the esophagus. This segment then breaks away from the main pulmonary anlage to form a CBPFM. CBPFMs should be considered in the workup of infants with respiratory distress and/or recurrent pneumonias. Patients with suspected pulmonary sequestration should undergo contrast studies to exclude a gastrointestinal communication.