A 2837 g male infant with an antenatal diagnosis of left CDH and TOF was born by cesarean section at 38 weeks of gestation. The Apgar scores were 5 at 1 min and 5 at 5 min. At the time of resuscitation, a 2.5 mm (ID) endotracheal tube could only be inserted just below the vocal cords. Therefore, congenital tracheal stenosis was suspected.
Chest X-ray confirmed a diagnosis of left CDH (Fig. 1). Echocardiography performed at 2 days of age showed only the right pulmonary artery. After stabilization, he underwent CDH repair at 3 days of age. The diaphragmatic defect was 6 × 4 × 5 cm. After reducing the herniated organs, including the small intestine, the left lobe of the liver, the stomach, and the spleen, the left lung could not be identified in the thoracic cavity. The defect was repaired using a GORE-TEX® Soft Tissue Patch (Thickness: 1 mm). At 7 days after birth, he was extubated successfully. However, he required re-intubation due to progressive respiratory distress at 9 days after birth. In addition to the tracheal edema caused by intubation, increased sputum after long-term intubation was considered to be the cause of obstruction. Enhanced CT revealed long segment CTS with left lung agenesis (Fig. 2). The lung segment in the left thoracic cavity on CT was the lower lobe of the right lung herniated into the left thoracic cavity, and the tracheal branch extending to the left was the right middle lobe branch, not connecting to the lung parenchyma. The tracheal stenosis was approximately 4 cm in length, extending from the upper trachea to the right main bronchus. In addition, it confirmed that the tip of the intubation tube was located just before the tracheal stenosis lesion. Bronchofiberscopy showed complete cartilage rings starting from just below the vocal cord.
At 24 days of age, veno-arterial extracorporeal membrane oxygenation (ECMO) was introduced due to life-threatening airway obstruction. In preoperative management, muscle relaxant was used once when breathing was unstable. At 28 days of age, slide tracheoplasty and palliative right ventricular outflow tract reconstruction (RVOTR) was performed with cardiopulmonary bypass (CPB) (body weight: 2826 g).
The operation was performed through a midline sternotomy plus collar incision. After thoracotomy, ECMO was switched to CPB using the aorta and right atrium cannulation. The ascending aorta, brachiocephalic artery, and trachea were dissected and encircled with vessel loops (Fig. 3). Following the establishment of cardiopulmonary bypass, palliative RVOTR and PDA ligation were performed. The brachiocephalic artery was transected temporarily to secure the upper field of slide tracheoplasty (Fig. 4a, b, Additional file 1). The tracheal stenosis was measured from the upper trachea to just above the carina (length: 40 mm, inner diameter: 2 mm). Transverse division of the trachea was performed in the middle of the stenosis (Fig. 4c). A longitudinal incision of 2 cm in length was made on the posterior side of the proximal trachea and the anterior side of the distal trachea. The two ends were slid to each other, and anastomosis was performed using 5–0 monofilament sutures. After reconstruction, a tracheal tube (ID: 3.5 mm, OD: 4.8 mm) was inserted near the tracheal bifurcation and placed so that the tip exceeded the anastomotic site (Fig. 4d). The brachiocephalic artery was reconstructed, because it was necessary to continue ECMO from the carotid artery after the operation. Then, CPB was successfully discontinued, followed by the reintroduction of ECMO. The operation time was 7 h and 36 min, and the amount of bleeding was 2197 ml (ECC: 1827 ml). As postoperative management, muscle relaxants were used for 14 h postoperatively, and ECMO was completed 7 days after the operation. During ECMO management, 3 days after surgery, a hyperechoic region appeared in the left middle cerebral artery region by head ultrasonography, which was cerebral edema due to cerebral infarction.
On the 17th postoperative day, he was successfully extubated. He was discharged at 5 months of age with home oxygenation therapy and continuous enteral nutrition through a nasogastric tube. Unfortunately, left side cerebral infarction was detected by postoperative head CT, which caused slightly delayed mental development. Six days after extubation, the infant no longer needed oxygen administration, but high-pressure treatment needed to be continued, and oxygen saturation easily decreased during awakening. Three months after the operation, the respiratory condition had settled down, and then preparations for discharge were started. At the time of discharge, it was necessary to continue Nasal High Flow, HOT therapy, and tube feeding at home. A 4-month-old chest CT image 3 months after the operation is shown. A 4-month-old chest CT image showed no left lung or trachea, and the right lung remained hernia from the dorsal side of the heart into the left thoracic cavity (Fig. 5), and bronchoscopy at 8 months of age showed no granulation, tracheomalacia, or re-stenosis after CTS construction (Fig. 6).
