Dear Editor,
Tracheoesophageal fistula (TEF) is a congenital condition characterized by an abnormal connection between the trachea and esophagus, frequently occurring alongside esophageal atresia, with an incidence of about 1 in 3 000 to 4 000 births globally. Up to 25% of neonates with TEF are also diagnosed with VACTERL association, which includes vertebral anomalies, imperforate anus, cardiac defects, renal agenesis, and limb abnormalities [1]. Tracheomalacia often accompanies esophageal atresia but can remain undetected due to external splinting forces. During surgery, when the fistula is divided, this external support is removed, potentially revealing tracheomalacia only after the operation has been completed [2]. We present a challenging case that illustrates the complexities of airway management in a neonate with a history of TEF repair who required surgery for suspected Hirschsprung disease. Informed consent for publication was obtained from the patient’s guardian.
A 10-day-old full-term neonate, weighing 2.6 kg, was scheduled for a diversion colostomy due to subacute intestinal obstruction with suspected Hirschsprung disease. The neonate had undergone TEF repair with esophageal atresia correction on day 2 of life via a right thoracotomy approach. The surgery was uneventful, and the patient was transferred to the neonate intensive care unit intubated and later extubated on post-operative day 1 without signs of respiratory distress.
Preoperatively, the neonate had stable vital signs and exhibited no signs of respiratory distress. Standard American Society of Anesthesiologists monitors were applied, and anesthesia induction was achieved using fentanyl (1 μg/kg) followed by titrated doses of propofol, with satisfactory bag-mask ventilation. Given concerns regarding airway collapse, minimal neuromuscular blockade was preferred. Atracurium (0.5 mg/kg) was cautiously administered for muscle relaxation for endotracheal intubation. Intubation was attempted using a 3-mm cuffed endotracheal tube under C-MAC video laryngoscope (Karl Storz SE & co. KG) guidance. However, post-intubation, we encountered significant ventilation challenges, including absent end-tidal CO2 and no air entry upon auscultation.
Re-evaluation with the C-MAC confirmed proper Endo Tracheal Tube (ETT) placement within the trachea, yet ventilation remained ineffective. Given the surgical history, the possibility of a residual TEF was considered; however, there were no signs of gastric insufflation. Meanwhile, the neonate developed bradycardia (heart rate: 56/min) and desaturated to 36%. Atropine was administered, and additional help was sought. A senior anesthesiologist reassessed the larynx and subglottic area for anomalies, cautiously withdrawing the ETT under C-MAC guidance. Ventilation was successfully established when the cuff of the ETT was positioned just inside the vocal cords. The tube was secured in this location, allowing the surgery to proceed uneventfully with stable hemodynamics.
Following the procedure, the ETT was replaced with an i-gel® (Intersurgical Ltd.) using Bailey’s maneuver. A fiber-optic bronchoscopy was subsequently performed, revealing a collapsing elliptical trachea, indicative of tracheomalacia (Fig. 1). The initial ETT placement may have inadvertently lodged in a blind pouch, or its bevel may have abutted the tracheal wall weakened by tracheomalacia, contributing to the ventilation difficulties. These issues became more pronounced after muscle relaxation, despite the neonate maintaining a patent airway without distress in the preoperative period.
Research suggests that TEF with esophageal atresia is associated with airway anomalies, including tracheomalacia. Tracheomalacia is characterized by weakness in the tracheal wall due to softening of supporting cartilage and hypotonia in myoelastic components. It is classified as either primary, occurring congenitally and potentially associated with other anomalies (e.g., cleft palate, choanal atresia, and esophageal atresia), or secondary, acquired due to external compression or prolonged intubation [3].
Patients with repaired TEF often present with respiratory and digestive system complications, including tracheomalacia, cyanotic and apneic episodes, and gastric regurgitation, which typically manifest later in life. When these patients require general anesthesia for subsequent surgeries, vigilance regarding potential respiratory complications is essential [4,5].
Key takeaways for airway management
• Anticipate Airway Challenges: Anesthesiologists should be aware of potential airway abnormalities in patients with a history of TEF repair, particularly tracheomalacia.
• Choice of Airway Device: Flexible fiber-optic bronchoscopy should be available, and alternative devices such as supraglottic airways (e.g., i-gel®) should be considered when difficulties arise.
• ETT Positioning: Maintaining the ETT tip at or just below the vocal cords may improve ventilation in cases of tracheomalacia.
• Effect of Muscle Relaxation: Neuromuscular blockade can exacerbate airway collapse in tracheomalacia by reducing the tone of the tracheal wall, which relies on myoelastic support for structural integrity. The loss of this tone further weakens an already compromised airway, making it more prone to dynamic collapse during ventilation. Clinicians should consider using minimal or no muscle relaxant if feasible and monitor airway patency closely, opting for spontaneous ventilation or alternative airway strategies when appropriate.
• Early Recognition and Intervention: If ventilation difficulties occur, withdrawing the ETT under direct visualization may help restore effective ventilation.
In conclusion, our experience highlights the need for adaptability, vigilance, and comprehensive preoperative preparation when anesthetizing neonates with a history of TEF repair. By anticipating airway-related complications and employing appropriate airway management strategies, anesthesiologists can optimize patient outcomes in this vulnerable population.
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