Tracheo-esophageal Fistula

Pediatric Surgery
This podcast discusses the work up, operative management, and follow-up management of tracheoesophageal fistulas.

Dr. Todd Ponsky is an associate professor of surgery and pediatrics and pediatric surgeon at Akron Children's Hospital.
Dr. Avraham Schlager is a pediatric surgeon at Akron Children's Hospital.
Dr. Daniel von Allmen is the surgeon-in-chief, Lester W. Martin chair of pediatric surgery, senior vice president of the surgical services, and professor at University of Cincinnati department of surgery.

Intro and outro track is adapted from "I dunno" by grapes, featuring J Lang, Morusque.
Artist URL: ccmixter.org/files/grapes/16626
License: creativecommons.org/licenses/by/3.0/

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Additional Content

Tracheoesophageal Fistula and Atresia (Daniel von Allmen, MD)

Diagnosis of tracheoesophageal fistula
  • History and Physical
    • History usually includes inability to pass nasogastric tube (NGT) and respiratory distress.
    • Patient usually has a distended abdomen on exam
    • Always attempt to personally place the NGT to confirm the history
  • Imaging
    • Inject air into NGT and obtain chest and abdominal X-rays to evaluate the location of the NGT and also assess the gas pattern of the gastrointestinal tract
    • Will usually see a proximal dilated esophageal pouch
      • If you do not see this, consider:
        • Incorrect diagnosis
        • A proximal fistula that is allowing decompression of the esophageal pouch
      • Consider a contrast study for further evaluation if you still have a high clinical suspicion
  • Additional workup for other congenital anomalies in VACTERL association
    • Preoperative echocardiogram is essential to evaluate for cardiac anomalies, most importantly, a right sided aortic arch as this will affect your operative plan
    • Renal ultrasound and spine imaging for a tethered cord may be done after management of the TEF

 

Managing a patient without respiratory compromise

  • If the patient does not have respiratory insufficiency (i.e. not intubated), then it is reasonable to delay the operation for a couple of days until all necessary resources (i.e. anesthesia support) are available
  • Significant delay in operative intervention will prevent proper colonization of GI tract and will result in soilage of the lung

 

Management of patient with respiratory compromise

  • Pathophysiology:
    • Respiratory compromise is usually secondary to poor lung compliance
    • In infants with poor compliance, more air is shunted into the GI tract, similarly to patients with large fistulas
  • Temporizing procedures may be necessary to stabilize the patient prior to definitive surgery. Examples are:
    • Bronchoscopy and using a Fogarty balloon to occlude the fistula
    • Gastrostomy tube (G tube) placement for decompression of the stomach
      • This may create lower resistance and shunt more air into the GI tract
    • Laparotomy with placement of vessel loop around the GE junction at the hiatus to be used as a Rumel tourniquet. This will also require placement of a G tube

Preoperative bronchoscopy
  • Assists in evaluating the location of the fistula, such as a proximal fistula, which will guide your operative intervention
  • Also be used to evaluate for a second fistula, which has an incidence of about 1%
  • It is also used to evaluate for a laryngeal cleft

Pitfalls
  • Proximal fistulas
    • Location may range from very proximal, which may be approached through a neck incision, to the carina
    • Endotracheal tube (ETT)
      • If fistula is at the carina, recommend leaving ETT high
      • If fistula is proximal to the carina, recommend placing ETT past the fistula. Auscultation will help evaluate if the ETT was placed inadvertently into the fistula
  • Right sided right sided arch
    • Approach TEF through left thoracotomy to avoid the aorta
    • If diagnosed intra-operatively, do not recommend converting to a right thoracotomy, as it is technically feasible to complete the operation with a good result
  • Duodenal atresia
    • If stable from respiratory standpoint, recommend fixing the duodenal atresia first. This would avoid fixing the TEF proximal to an obstruction, which may compromise the esophageal anastomosis
    • Repair of both atresia is usually performed as a staged operation, though you may consider fixing both anomalies at the same time if the patient is stable and there is good anesthesia support

Open operative technique
  • Position the patient in left lateral decubitus
  • Incision is a right posterolateral thoracotomy
    • Muscle sparing thoracotomy is an option to salvage more muscle, but it may make adequate exposure difficult to obtain
  • Extrapleural approach
    • After dissecting through the intercostal muscles, Dr. von Allmen uses a right angle with the heel on the ribs to separate the muscles and expose the extrapleural space
    • Extrapleural approach is preferred to transpleural because it avoids soilage of the pleural space if there is an anastomotic leak
  • Azygous vein is used as a guide to the location of the fistula
    • Mobilize the pleura posteriorly to gain exposure to the azygous vein
    • Divide the azygous to gain better access to the fistula
  • Identify the esophageal segments
    • Mobilizing the pleura to the apex of the lung will allow easier access to the proximal pouch of the esophagus
      • Having the anesthesiologist push on the NGT may help identify the proximal pouch
    • Identify the distal esophageal segment and control with a vessel loop
    • Dissect up proximally to identify the location of the fistula
  • Fistula ligation
    • Usually performed by suture ligation in the open technique
    • Clips, ligasure, hook electrocautery and 3 mm sealer are alternative methods mainly used thoracoscopically
  • Mobilization of esophagus from trachea
    • Use a combination of blunt dissection and blade electrocautery
    • Stay on esophagus to avoid getting into trachea
    • Injury to the trachea
      • Usually able to close primarily
      • If unable to do so, sleeve resection and primary repair is an option as the trachea is very mobile
        • Recommend placement of pericardium or other autologous tissue between the trachea and esophagus
  • Mobilizing the esophagus to gain adequate length
    • Mobilization of the proximal esophagus will give more length than the distal esophagus
    • Distal esophagus may be mobilized laterally down to the diaphragm. If mobilizing medially, then need careful dissection to avoid the blood supply
  • Suture preference for anastomosis
    • Recommend absorbable or monofilament suture, such as PDS
    • Some surgeons use silk and vicryl
  • Techniques used to manage long gap esophageal atresia
    • Myotomy
      • This may create a more dysfunctional segment of esophagus
    • Reverse flap
    • Elongating techniques
      • Uses suture to stretch the esophagus. Pressure may also act as a stimulus for growth
        • Will usually be able to bring the esophageal ends together after 1-2 weeks
      •  Foker procedure
        • Sequential tensioning of sutures to stretch the esophagus
        • Requires prolonged ICU stay with intubation and paralysis of the patient
        • Recent study in the Journal of Pediatric Surgery found the Foker procedure was successful in 96% of patients with primary cases, and only 68% of patients who had previous repair
          • Bairdain S, Hamilton TE, Smithers CJ, Manfredi M, Ngo P, Gallagher D, et al. Foker process for the correction of long gap esophageal atresia: Primary treatment versus secondary treatment after prior esophageal surgery. J Pediatr Surg 2015;50:933–937. http://www.jpedsurg.org/article/S0022-3468(15)00181-5/abstract
      • "Internal" Foker procedure
        • Involves tacking the sutures to the prevertebral fascia without subsequent tensioning
        • This method does not require postoperative paralysis of the patient
      • May also perform this thoracoscopically by bringing out the sutures out through the trocar sites and placement of them on tension

Thoracoscopic approach
  • Lung isolation techniques
    • Oscillators may have decreased lung inflation compared to other modes
    • Lung inflation usually can be overcome by CO2 insufflation
      • Often results in desaturation initially from shunting
    • May consider single lung ventilation
    • Adding an extra port to function as a lung retractor is reasonable
    • Rotating patient past 90 degrees will allow gravity to assist with keeping the ipsilateral lung away from the operating field
  • Port placement
    • Usually performed by three ports that are triangulated. The camera is in the center, a posterior port placed inferiorly and anterior port placed superiorly
    • Usually use 3mm instruments
  • Azygous vein may be ligated using a sealer
  • Ligation of fistula may be performed using clips
    • Wait to cut it until after placement of your first suture to prevent the distal pouch dropping away from your field
  • Mobilization of proximal esophagus
    • Thoracoscopy allows better visualization of the proximal esophagus
    • Grabbing the proximal esophageal pouch and twirling it ("Spaghetti” method) is a technique used to show the dissection plane
  • Creation of anastomosis
    • More technically difficult than open and requires a lot of experience
    • Must incorporate mucosa on every stitch
    • Pearls
      • Place a stay stitch on each end of the anastomosis and pull out of the chest.. When you hold up the two ends, it will line up your anastomosis and allow you to see your lumen
      •  If using a knot pusher, recommend use monofilament so you can easily push down the knot and avoid tearing the esophagus.  If performing intracorporeal knots, then braided suture can be safely used

Drain placement
  • Drain is placed in the extrapleural space
  •  New data shows that a trans-anastomotic drains may result in higher stricture and leak rates

Postoperative management
  • Extubation plan
    • If had good respiratory function going into the OR, then early extubation, even in the OR, should be considered
    • Early extubation is preferred to limit positive pressure from the ventilator on the tracheal repair
  • Esophagram
    • Usually performed post operative day 5-7
    • If negative, may remove chest drain and start oral feeds
    • There is no indication for repeat imaging if patient is asymptomatic

Management of a stricture
  • If seen on postoperative esophagram
  • Post operative esophagogram usually shows a narrowing because the proximal pouch is more dilated than the distal pouch
    • As long as it is patent with contrast easily passing through, recommend close monitoring and dilation after several weeks if still needed
  • Recommend dilation using a balloon.
  • Repeat esophagram after 1-2 weeks to reevaluate and dilate again as needed

Management of an anastomotic leak
  • If the leak is small and the patient is clinically stable, recommend leaving the drain and monitor clinically. The majority will close spontaneously.
  • Repeat esophagram every week to evaluate the leak.  If repeat esophagram is negative, may remove the chest drain at that point.
  • Indications to go to the operating room are if the patient is unstable and the leak is large and uncontained with a possible fistula and pneumothorax
  • A leak will increase the incidence of a stricture

Postoperative Follow-up

  • Require regular follow-up until the patient is asymptomatic and eating well

Long-term complications
  • Strictures
    • Patients often present with obstructions from food boluses and may need serial dilations
  • Reflux
    • Unknown malignant risk of long term reflux
    • Most patients require anti-reflux medications after a TEF repair
  •  Indications for anti-reflux surgery
    • If the patient is requiring serial dilations (2-3 times) with short periods of time between each dilation
    • Poorly controlled reflux from their esophageal dysmotility
    • A loose Nissen fundoplication is often performed

Management of pure esophageal atresia
  • Requires placement of a G tube initially
  • Gauge the length of the atresia by placing a radiopaque catheter through the G tube and the NG tube
  • Repeat this imaging to assess how close the ends comes together over the next 4-6 weeks as the patient grows
  • Plan primary repair once the esophageal ends are within 2-3 vertebral bodies

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