右主気管支が食道に開口した Bronchopulmonary Foregut Malformation の1例

概要

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Bronchopulmonary foregut malformation (BFM) is an anomaly which is caused by an anomalous embryology of the foregut. We experienced a case of BFM in which the right main stem bronchus was communicating with the esophagus. This 16-day-old male infant showed tachypnea from birth. Respiratory sound was not audible in the right lung field. Esophagogram revealed a bronchus-like structure arising from the esophagus and spreading to the right hemithorax. Esophagoscopy revealed a fistula on the right posterior wall of the esophagus. Operation was performed on the 23rd day after birth. The right lung consisted of only one lobe. It communicated with not the trachea, but with the lower esophagus by a fistulous tract that seemed to be the right main stem bronchus. Blood supply flowed from the right pulmonary artery. The right lung was removed. Pathological examination revealed the presence of mature bronchioles, alveolars and vessels in the right lung tissue. The lumen of the tract communicating with the esophagus was lined with stratified columnar epithelium and the cartilage was present in the fistulous wall. Since the operation, the patient has had recurrent pneumonitis, and is diagnosed as having right pneumonectomy syndrome.
日本小児外科学会の論文
1991-06-20