Repair of tracheoesophageal fistula (TEF)
ETT ± Spontaneous Ventilation, Mainstem Intubation, or Fogarty Catheter
|Lines and access||
PIV, Arterial Line
Standard, 5-Lead ECG, Temperature, Pulse Oximeter, NIBP or ABP
|Primary anesthetic considerations|
Fistula Type, VACTERL Association
PPV vs. NPV, Paralysis vs. No Paralysis, Tolerance of Thoracoscopic Insufflation
Transferring Care While Intubated
Tracheoesophageal fistula (TEF) is a congenital anomaly seen in the neonatal period in which a patient's trachea and esophagus fail to septate properly. TEF occurs in approximately 1 in 3,000 live births. Prenatal ultrasound can show an absent gastric bubble and polyhydramnios. In the neonatal period, signs and symptoms of TEF include respiratory distress with feeding, regurgitation, and choking. Diagnosis is confirmed by failing to pass an orogastric tube, with radiography showing tube coiling in the upper airway, increased gastric and intestinal air, and possible pneumonitis. Surgical repair is the only definitive therapy. Anesthesia concerns are primarily related to airway management.
TEF comes in five different anatomical configurations. The most common is a "Type C" fistula, seen in ~85% of patients, consisting of proximal esophageal atresia with a fistula between the trachea and the distal esophageal segment. Other configurations include:
Type A: Esophageal atresia without any fistula connection between the trachea and esophagus.
Type B: Esophageal atresia with a fistula between the trachea and the proximal esophageal segment.
Type D: Esophageal atresia with two fistulae– one between the trachea and the proximal esophageal segment, and one between the trachea and the distal esophageal segment.
Type E: Fistula between the trachea and esophagus without esophageal atresia (also called an "H-Type Fistula").
The position of the fistula can have a significant impact on airway management.
When TEF is diagnosed, workup must also be initiated for associated congenital anomalies referred to as the "VACTERL" Association. Diagnosis requires the presence of at least two of the following conditions: Vertebral anomalies (seen in 60%-90% of VACTERL diagnoses), Anorectal malformations (55%-90%), Cardiac anomalies (40%-80%), TEF (50%-80%), Renal anomalies (50%-80%), and Limb anomalies (40%-55%). Presence or absence of cardiac anomalies, including cyanotic congenital heart defects, situs inversus, vascular anomalies or arrhythmias, has the biggest impact on overall survival and anesthetic management.
Surgery can be performed via an open or thoracoscopic approach. Thoracoscopy avoids many of the complications of the open approach, such as development of scoliosis or shoulder glide weakness. Thoracoscopy has also been associated with longer operating times, but shorter time to extubation and shorter time to first enteral feeding, without significant difference in complication rates. The major steps are the same for both approaches. The distal and proximal esophageal segments are identified, as well as the trachea and fistula. The azygos vein and vagus nerve are also identified, and care is taken to avoid damage to these structures. The fistula is clipped or suture ligated proximal to the trachea. Importantly, the fistula may not be divided following closure to prevent contracture of the connected esophageal segment. At this point, the proximal and distal esophageal segments are opened. A nasogastric tube is placed across them under direct visualization to guide the anastomosis and ensure patency.
|Airway||Review imaging prior to patient evaluation to confirm the fistula type. There are several options for intubation, including general endotracheal intubation, or intentional main stem intubation below the level of the fistula. Plan should be made based on the number of fistulae, whether they are proximal or distal, and relative position to the carina. Bronchoscopy may also be performed immediately prior to surgery.|
|Cardiovascular||VACTERL anomalies may be present, including cyanotic CHD, situs inversus, right sided aortic arch, arrhythmia, or others. These should be screened for prior to the procedure, and considered when making an anesthetic plan.|
|Pulmonary||Respiratory distress may be present to varying degrees. Most fistula types result in increased gastric air, increasing abdominal pressure and difficulty ventilating. If the patient is currently intubated, confirm vent settings, assess ETCO2, and get a VBG to determine adequacy of ventilation.|
|Gastrointestinal||Confirming the fistula type by radiography is an important pre-operative step. This is typically done by attempting to pass an orogastric tube (commonly, a Repogle tube) and taking a chest and abdominal X-ray. Coiling of the tube in the proximal esophagus indicates the presence of atresia, while the presence or absence of air in the stomach and intestines.|
|Renal||Renal anomalies may be seen as part of the VACTERL association. Preoperative chemistries should be evaluated.|
|Musculoskeletal||Vertebral anomalies may be present in the VACTERL association. Cervical vertebral malformations, abnormal curvature, and tethered cord syndrome may be present, and should be considered prior to intubation.|
Labs and studies
- Chest and abdominal X-rays aid in diagnosis and can help identify fistula type as described above
- Echocardiography should be considered, but may not be necessary in the absence of abnormal cardiac exam findings.
- CBC, CMP, and Blood Gasses should all be considered pre-op.
Monitoring and access
- Blood Pressure: Invasive blood pressure monitoring may be indicated for patients with associated cardiac anomalies.
- Pulse Oximetry and EtCO2: Important for monitoring adequacy of ventilation.
- Telemetry Monitoring
- Temperature Monitoring
- Inhalational inductions are typically used, favored for their hemodynamic stability.
- Propofol or Dexmedetomidine should be on hand, and may be used if rapid IV induction is needed.
- Muscle relaxing agents (eg. Rocuronium) may be used depending on the chosen airway management strategy.
|Strategies for airway management (as described in Broemling and Campbell)|
|Spontaneous Ventilation||Positive pressure ventilation increases the risk of gastric insufflation and ventilatory compromise. Physiologic negative pressure ventilation lowers the risk of increasing airflow through the fistula. In patients with good pulmonary compliance or demonstrated easy masking without gastric distention, spontaneous ventilation may be tried, knowing standard intubation and PPV is a viable backup.|
|Main Stem Intubation and One-Lung Ventilation||This method allows for collapse of the lung in the operative field during fistula ligation. The tube can often be retracted to the trachea following fistula ligation. Main stem intubation typically requires increased FiO2 and flow rates to maintain adequate oxygenation.|
|Distal-Fistula Intubation||Intubation distal to the fistula can allow for bilateral ventilation with positive pressure for neonates with respiratory failure. Caution must be used to ensure the tube does not migrate into a main stem bronchus. Migration of the ETT into the fistula may also occur, and can have extremely morbid outcomes.|
|Fistula Occlusion||Fistula occlusion via Fogarty catheter can also be considered, but is difficult to execute and may dislodge, occluding the central airways.|
Positioning is lateral recumbent, typically with the right side elevated ~30º for thoracic access and proper positioning of the lung relative to the mediastinum.
Maintenance and surgical considerations
- Inhaled agents are typically used for maintenance of anesthesia.
- Insufflation for thoracoscopy may lead to desaturations and decreased venous return. The patient should be converted to an open procedure in that case, with use of additional anesthetic and pain control as indicated. This may also indicate a need for neuromuscular blockade and positive pressure ventilation if not already in use.
- Following ligation of the fistula, positive pressure ventilation may be initiated if needed without concern for gastric distention.
- Extubation depends on ventilatory needs post op, as well as concern for laryngomalacia or tracheomalacia, injury to the recurrent laryngeal nerve, or suspicion for other ventilatory complications. Patient may be transferred post op while still intubated.
- Patients may remain intubated following the case, and will need to be monitored in a Neonatal Intensive Care Unit as ventilatory support is weaned.
- Post-op enteral feeding may be administered through a trans-anastomotic nasogastric tube. This may shorten time on TPN, however subsequent reviews have found an increased risk of esophageal stricture and no significant reduction in anastomotic leak.
- Opioid sparing techniques, such as regional anesthetics in the thorax, should be used with caution due to immature hepatic metabolism of these agents.
Complications were noted in the cited review in as many as 62% of cases, with the most common being:
- Esophageal Stricture (42%)
- Anastomotic Leak (23%)
- Vocal Cord Injury (7%)
- Recurrent Fistula (5%)
- Anastomotic dehiscence (2%)
These may lead to additional consequences, such as chylothorax, empyema, pleural effusion, pneumothorax, and issues with growth and development.
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