Tracheoesophageal Fistula (TEF) Repair

TRACHEOESOPHAGEAL FISTULA (TEF) REPAIR

Disease Summary

• TEF is an abnormal connection between the esophagus and trachea. It is a relatively common congenital disease of the respiratory tract that in the US has an incidence of 1 in 3500 to 1 in 4500.

• Pathogenesis

  • TEF results from a defect in the lateral septation of the foregut into the trachea and esophagus. The fistula is thought to originate from a branch of embryonic lung bud that fails to begin branching due to defective epithelial mesenchymal interactions. TEF symptoms most often present in infants immediately after birth with choking, drooling, respiratory distress and the inability to feed. When gastric contents are refluxed through the TEF it results in aspiration pneumonia which is a significant cause of morbidity.

• Associated Anomalies

  • TEF most often occurs alongside esophageal atresia (95% of cases). Associated anomalies are present in about 50% of cases, most commonly with VACTREL associations. VACTREL associations are vertebral anomalies, anal atresia, cardiac defects, tracheoesophageal fistula, renal defects and limb defects. The high prevalence of congenital heart disease associated with TEFs necessitates preoperative electrocardiogram and echocardiogram in all patients planning on receiving TEF surgical repair. Regarding vertebral anomalies, it is recommended that neonates with sacral dimple receive lumbar ultrasound. Prematurity has an incidence of 30% in TEF, which leads to poor lung maturity and can result in ventilatory compromise, an important implication in anesthesia airway management.

Case Planning

• Specific or Unique Room Set-Up Requirements

  • Airway

    • Prior to securing the airway, the surgeon will perform bronchoscopy to evaluate the site of the fistula.

      • The patient should be kept breathing spontaneously for bronchoscopy

    • After bronchoscopy, the trachea should be intubated.

    • Care should be taken to avoid vigorous positive pressure ventilation via a mask as this may insufflate the stomach via the fistula and impair ventilation.

    • Optimally, the cuff of the ETT should be placed below the fistula to ensure fistula ventilation does not occur

      • Consider a micro-cuffed ETT (if available)

      • If the location of the fistula is so distal that the ETT cuff cannot be maneuvered past it, mainstem intubation may be required and/or maintenance of spontaneous ventilation until the fistula is ligated

  • Drugs/Infusions

    • Various medications may be used to facilitate bronchoscopy

      • Volatile anesthesia

      • Ketamine

      • Propofol (infusion/bolus)

      • Dexmedetomidine

    • Consider steroids to prevent airway edema

    • Non-depolarizing muscle relaxation may be considered if the airway can be secured distal to the fistula

    • Narcotic analgesia

  • Monitors

    • Standard ASA monitors

    • Arterial access is not mandatory but may be paced at the discretion of the attending anesthesiologist

  • Blood Availability (if indicated)

    • Consider T+C x 1 unit

  • PICU Bed Availability

    • These patients frequently present from NICU

    • Most patients remain intubated post-operatively

      • Discuss with surgeon, but usually they would like to make absolutely sure the child will not require emergent reintubation which could traumatize the fistula

Anesthetic Considerations

• Induction

  • Most patients present with IV/Central access from the NICU

  • Consider a mask induction to maintain spontaneous ventilation and avoid positive pressure mask ventilation

    • Consider avoiding nitrous oxide

      • 100% FiO2 will increase the time to desaturation in the event of emergency

      • Nitrous oxide may collect and expand the stomach preventing effective ventilation

  • Discuss emergency plans with the surgeon

    • If mask ventilation is required and gastric insufflation impairs effective ventilation, emergency percutaneous gastric decompression may be required

  • Following bronchoscopy, the trachea maybe intubated with direct laryngoscopy or using bronchoscopy

    • ETT cuff position distal to the fistula should be confirmed

    • Have a high degree of suspicion that the ETT may intubate the fistula leading to ventilation of the stomach

  • Consider non-deoplarizing muscle relaxation after controlled ventilation is achieved

    • Attempt to maintain spontaneous ventilation if the fistula cannot be adequately isolated

• Positioning

  • Left Lateral

  • An underbody warming blanket should be used

• Maintenance

  • Maintenance of Anesthesia

    • Hemodynamic/Physiologic goals

      • Spontaneous ventilation should be maintained until the trachea is intubated distal to the fistula

      • As most of these patients are neonates the goal MAP should be the patient’s gestational age

        • Ex. A 1 day old born at 37 weeks should have a MAP of 37 or above

    • Anesthesia may be maintained with a combination of:

      • Volatile anesthetic

      • Narcotics (such as fentanyl)

      • Non-depolarizing muscle relaxation

  • Surgical Considerations (such as neuromonitoring, muscle relaxation, anticipated blood loss)

    • Plan for post-op ventilation to prevent emergency intubation and accidental disruption of the fistula

• Emergence/Disposition

  • Return to NICU intubated with adequate analgesia and muscle relaxation on board

• Post-Op Pain Management

  • IV narcotics

  • Acetaminophen

  • Due to NICU unfamiliarity with their management, continuous caudal and epidural catheters are NOT recommended at this time

• Case-Specific Complications

  • Aspiration

    • A passage exists between the GI and respiratory tracts

      • Gastric secretions may easily reflux through the fistula

    • If esophageal atresia exists, oral secretions have no means of evacuation and may reflux into the trachea

  • Difficult ventilation

    • The TE Fistula represents a low-resistance path for positive-pressure ventilation to insufflate the stomach

      • The surgeon should be available to decompress the stomach percutaneously in the event of loss of ventilation

    • Even after intubation distal to the fistula, manipulation of the patient may lead to intubation of the fistula and loss of pulmonary ventilation

      • Once the fistula is ligated, there is still a small blind pouch

        • If the ETT migrates into this pouch, ventilation may become impossible

          • The ETT should be pulled back until effective ventilation is restored