Results
Five neonates with Gross type-C EA/TEF with compromised respiration were enrolled. Table 1 shows the summaries of their clinical characteristics and associated management. Four neonates had body weights of less than 2,500 g and two neonates had gestation age less than 37 weeks. All of them developed respiratory distress immediately after birth and had received nasal bi-prongs PPV supports before the FE. In four inborn neonates, these FE interventions were performed within six-hours of age, while Case 5 was referred from another medical center and the FE performed on her third day of life (DOL).
After diagnosis and measurements were confirmed with FE-NIV-SPI, NTFG tube was immediately placed as early therapeutic intervention in all neonates. In three neonates, FE-NIV-SPI also aided in nasal ETT intubations, with the presence of the NTFG tube, by safely guiding the ETT tip passed over the fistula opening and appropriately positioned just above the carina. Both NTFG tube and ETT were well visualized as wedging into the vocal cords. The mean procedural duration of the whole FE-NIV, including both diagnostic and therapeutic, was 13.6±4.5 minutes.
Afterward, all neonates received regular gastric feeding and intermittent gastric decompression via the NTFG tubes. Their vital signs and respiratory statuses were stable prior to surgical correction of EA/TEF, except for Case 4, where the patient required urgent cardiac surgery due to associated cardiovascular comorbidity. During correction surgery of EA/TEF, after confirming the fistula sites in all patients, surgeons removed the NTFG tubes just before ligation of the fistula. The mean duration of NTFG tube placement was 11.4±18.2 days. All neonates survived and had follow-up for more than 18 months. There were no complications associated with the FE-NIV-SPI and NTFG tube placement including air leakage, airway bleeding, laceration or stenosis. Three neonates (Case 1, 3 and 4) received 2 to 4 courses of laser therapy and balloon dilatation due to mid-esophageal stenosis at the anastomosis sites.
Case presentation (Case 4)
A full-term male neonate, birth weight 2,390 g with prenatal polyhydramnios and echocardiographic diagnosis of transposition of great artery, was born by spontaneous delivery. Respiratory distress soon developed after birth and he underwent nasal ETT (3.0 mm inner diameter) intubation with mechanical ventilation settings: fraction of inspiratory oxygen 50%, PEEP 6 cmH2O, PIP 20 cmH2O and rate of 20 per minute. Unfortunately, he gradually developed obvious stomach distention and failed multiple insertion of gastric tube, therefore presence of EA/TEF malformation was suspected.
Diagnostic FE-NIV-SPI was performed at age of 5 hours old with ETT extubation. A folded esophageal tube was seen at hypopharynx (Figure 2) with severe (>95% collapsed) TM in mid-tracheal which occluding scope view of the distal trachea. After applying 2 to 3 seconds of SPI, the collapsed portion gradually opened and a large fistula with width of 6 mm (Figure 3) was revealed at the posterior wall, 8 mm proximal to the carina level, while estimated the length of TM about 15 mm. The scope then advanced through the fistula, down into the lower esophagus and found stomach cavity, no obvious structure of sphincter over gastroesophageal junction was noted. After completing tracheal, fistula and low esophageal assessment, the scope was drawn back to pharynx and inserted into the inlet of esophagus. With aid of SPI (2 to 5 seconds), a gradual dilated esophageal blind pouch of about 2 cm length was disclosed. Therefore, FE-NIV-SPI technique could assist the accurate diagnosis of EA/TEF and associated anatomic relationships.
After confirming the diagnosis, therapeutic FE-NIV-SPI was carried out. An 8 Fr. suction catheter was selected as the NTFG tube with appropriate insertion depth to the stomach marked. With aid of Magill forceps, the catheter was inserted nasally, through the vocal cords and into the tracheal lumen (similar to nasal ETT intubation). The scope (ENF-V3, 2.6 mm) then advanced through the nose and wedged into the tracheal lumen (Figure 4) alongside the NTFG tube. Under FE guidance and SPI (2 to 3 seconds), endoscopist manipulated the tube into the wide-open fistula orifice smoothly, and propelled it forward into the gastric cavity. The endoscope itself could also advance into the low esophagus, alongside the tube, and confirmed the tube’s tip located inside the gastric cavity.
After placement of NTFG tube, a nasal ETT with internal diameter of 3.0mm was wedged alongside of the NTFG tube through the vocal cords with aid of Magill forceps (Figure 5). Then, a slim flexible endoscope (OD 1.8 mm, LF-P, Olympus) was cannulated through the ETT lumen and assisted in guiding the tip of ETT passed over the TEF till it reached just above the level of carina. Finally, the nasal ETT was fixated and connected to mechanical ventilator while the NTFG tube was used for feeding and gastric decompression.
Cardiac surgery was performed on the 10th DOL with a complicated course. On 44th DOL, cardiac function finally stabilized and a primary one-stage repair of the EA/TEF was carried out. During operation, the NTFG tube assisted in identifying the fistula location before its removal. Postoperative clinical course was uneventful, except for the esophageal stenosis, where he received two courses of laser therapy to cut off the fibrotic scare and four courses of balloon dilatations. These therapeutic FE all performed under the FE-NIV approach.13,16 At the time of this article writing, he is two years old with excellent respiratory function and oral feeding status.