外科的治療が考慮されたが，気管支食道起始が肺無形成の対側で，手術対象が残存肺になること，気管狭窄部位が長いうえに，右主気管支もスリット状であることから危険度が高いと判断された。
そのため内科的管理，即ち十二指腸チューブの挿入による誤嚥の予防，鎮静と早期抜管による気道の安静に努めた。しかし，肺炎を反復し呼吸状態は徐々に悪化した。日齢128 に右気管支食道起始切離術が行われたが，術後15日に呼吸不全のため死亡した。仮に気管再建術，罹患肺の切除が不可能でも，早期の食道と気管の交通の閉鎖が予後を改善した可能性が考えられた。
Contrast-enhanced multidetector row computed tomography（CT）performed on the first day of life demonstrated tracheal stenosis and a communication between the lower esophagus and right lower lung. Additionally, the left pulmonary parenchyma, blood vessels, and bronchial tree beyond the tracheal bifurcation were completely absent. Contrast medium instilled into the esophagus outlined the bronchial tree of the right middle and lower lobe.
Most of the reported patients have undergone desperate attempts at tracheal reconstruction with few successes reported. The outlook of the patients with critical tracheal stenosis involving long segments associated with unilateral pulmonary agenesis and BPFM was considered to extremely poor. To make matters worse, her anomalous bronchi arising from the esophagus connected to contralateral lung, so that we suspended a surgical intervention. And then, to make attempt to manage the deterioration of the extant lung, we used a nasoduodenal tube for feeding to avoid aspiration, and put her under sedation for airway rest. However, pneumonia recurred in the right lower lung field and developed life-threatening respiratory distress. Whereas, it was thought that she would not survive attempted repair, the right communicating duct was surgically ligated 4 months after birth. The fifteen days after the operation, however, the infant died from unexpected pulmonary hemorrhage.
