Hugh G. Auchincloss and Douglas J. Mathisen
INDICATIONS/CONTRAINDICATIONS
Acquired nonmalignant tracheoesophageal fistula (TEF) most commonly arises as a complication of endotracheal intubation. Pressure exerted by the endotracheal tube cuff on the tracheal wall leads to circumferential necrosis and potential for fistulization. The presence of a nasoenteric feeding tube greatly increases this risk. High-volume, low-pressure endotracheal tube cuffs and avoidance of prolonged nasoenteric tube placement have reduced but not eliminated this complication. Nonmalignant TEF may also occur secondary to blunt or penetrating trauma, in the setting of mediastinal inflammatory or granulomatous disease, or as a late complication following esophagectomy or laryngectomy (Table 38.1).
The presentation of TEF ranges considerably. Some patients may develop overwhelming pulmonary sepsis and inability to wean from the ventilator; others may present with chronic cough and aspiration years after intubation. In all cases the presence of TEF mandates surgical repair. Once formed there is little hope of the fistula closing with conservative measures alone, and the natural history of the condition is one of the intractable pulmonary complications and death. Continued mechanical ventilation is considered a relative contraindication to repair because positive pressure ventilation postoperatively is a risk factor for failure of the tracheal repair. However, in some patients the fistula represents the major impediment to ventilator weaning, and so, repair must be considered under suboptimal conditions.
Several approaches to repair of nonmalignant TEF have been described. The most conservative approach is a two-stage repair involving tracheal resection and reconstruction along with esophageal diversion, followed by interval esophageal reconstruction. Hermes Grillo demonstrated that similar results could be achieved with a single-stage tracheal resection and reconstruction and primary esophageal repair with muscle flap interposition. We believe this approach remains the gold standard for management of nonmalignant TEF. Others have proposed primary repair of both trachea and esophagus. This may be adequate for small fistulae but leads to unacceptably high rates of recurrence when the fistula is large. The increasing popularity of tracheal and esophageal stents in the field of thoracic surgery has led some to propose their use in the management of nonmalignant TEF. We feel that luminal stents are inadequate in that they do not address the underlying fistula and have potential for considerable harm by recreating the conditions under which the fistula formed leading to potential extension of the defect, and by making definitive repair more difficult. However, for malignant TEF—which usually occurs in the setting of aggressive cancer with a poor prognosis—luminal stents should be employed to achieve the goal of palliation.
TABLE 38.1 Patient Characteristics and Etiology of TEF
PREOPERATIVE PLANNING
Preparation for operative repair of nonmalignant TEF focuses on two things: Control of pulmonary sepsis by preventing further aspiration, and optimizing nutritional status. In the patient with a tracheostomy in place the former can be accomplished by placing a long tracheostomy tube with the cuff inflated distal to the fistula. Placement of a gastrostomy tube for drainage and a jejunostomy tube for feeding ensures that the patient will receive enteral nutrition with minimal aspiration risk. This approach excludes the fistula while improving the patient’s overall clinical condition. Every attempt should be made to wean a patient from the ventilator before undertaking operative repair.
All patients undergoing repair of a TEF should undergo preoperative bronchoscopy and esophagoscopy. Understanding the anatomy and precise location of the fistula as well as the condition of the uninvolved trachea and esophagus is a crucial part of operative planning. Active tracheal inflammation is often the cause to delay elective tracheal resection and reconstruction for management of tracheal stenosis; however, in the case of TEF it is unlikely that tracheal inflammation will subside completely and delaying repair for this reason would be inappropriate.
SURGERY
Repair of a nonmalignant TEF is a complex operation that should only be performed by an experienced thoracic surgeon at a high-volume center. Issues to emphasize include: Involvement of an experienced anesthesia team, complete resection of all involved trachea, two-layered longitudinal repair of the esophageal defect, and interposition of a suitable muscle flap between the repaired esophagus and the tracheal anastomosis.
Anesthesia
As with all airway surgery, TEF repair requires the involvement of a skilled anesthesia team. This is particularly true during induction of anesthesia and during periods when ventilation may be temporarily interrupted. The surgeon should be present during induction and flexible and rigid bronchoscopy should be immediately available. The safest method of induction is to breathe the patient down with an inhaled agent such as sevoflurane, followed by fiberoptic intubation with the endotracheal tube cuff inflated distal to the fistula. If the patient requires bag-mask ventilation prior to intubation great care is taken to avoid excessive positive pressure as this will distend the stomach and increase the chance of aspiration. During the operation anesthesia is maintained with intravenous agents to prevent wakening during periods when ventilation is interrupted.
Positioning
The patient is placed in the supine position with the head slightly elevated and the neck partially extended with a thyroid bag. Overextension of the neck may shorten the distance between the trachea and the manubrium and make dissection difficult. After the surgical site is prepped and draped, sterile equipment for cross-table ventilation is brought on to the field and prepared for use.
Technique
The majority of TEFs can be approached through a low collar incision (Fig. 38.1). If necessary a partial sternotomy may be performed to provide exposure to the distal trachea (Fig. 38.2). When a tracheostomy is present the incision should incorporate the stoma (Fig. 38.1). Additional exposure can be obtain by extending the collar inversion and dividing the manubrium. Subplatysmal flaps are created extending laterally to the anterior border of the sternocleidomastoid and cephalad and caudad sufficient to encompass the superior and inferior margins of the tracheal resection. The strap muscles are defined and dissected laterally to expose the thyroid isthmus. The isthmus is then divided and suture ligated with the cut ends retracted laterally. A pretracheal plane is developed cephalad and caudad using blunt dissection. At this point it may be useful to mark the precise location of the fistula by introducing a 25-gauge needle into the trachea under bronchoscopic vision. This requires that the endotracheal tube be temporarily moved proximal to the fistula. Once the site of the fistula is known the trachea is dissected circumferentially in this area. The dissection should remain as close to the trachea as possible to prevent injury to the recurrent laryngeal nerves. The dissection should be carried out cephalad and caudad to the site of anticipated tracheal division with care taken to prevent excessive devascularization (Fig. 38.3). If a stoma is present the decision must be made whether it can be reasonably incorporated into a tracheal resection. The trachea is then divided sharply, the endotracheal tube is withdrawn, and the distal trachea is intubated for cross-table ventilation (Fig. 38.3). With the fistula now exposed a plane can be developed between the membranous trachea and the esophagus. The esophageal mucosa is debrided as necessary and a two-layer longitudinal closure is performed using interrupted inverting 4-0 silk sutures for the mucosa with knots lying intraluminally and interrupted 4-0 silk sutures for the muscular layer (Fig. 38.4). The repair is performed over a nasogastric tube to prevent narrowing of the esophageal lumen. A pedicled strap muscle is used to reinforce the repair anteriorly and fixed in place using interrupted silk sutures (Fig. 38.5).
Figure 38.1 Incision for TEF repair.
Figure 38.2 Partial sternotomy gives excellent distal exposure.
Figure 38.3 Division of trachea with cross-table ventilation and exposure of fistula.
Figure 38.4 Two-layer esophageal repair.
Attention is now turned to the tracheal repair. If the fistula is small there may be no need for tracheal resection. If there is a large defect, coexistent tracheal stenosis, or a stoma within reasonable distance of the fistula then a short segment of trachea may be resected. If there is long-segment unresectable tracheal stenosis then the trachea should be closed over a T-tube (Fig. 38.6). Vicryl stay sutures are placed in the midlateral trachea proximally and distally. Interrupted 4-0 vicryl sutures are then used for the tracheal anastomosis beginning with the membranous wall and progressing anteriorly as described elsewhere. The sutures remain untied and placed such that the eventual knot will be extraluminal. Once all sutures are in place the cross-table ventilation is stopped and withdrawn from the distal trachea and the endotracheal tube is advanced distal to the anastomosis. The thyroid bag is deflated to allow for neck flexion. The sutures are tied and the anastomosis is tested for a leak by deflating the endotracheal tube cuff and ventilating the patient. If a leak is present it should be repaired with simple interrupted 4-0 vicryl sutures. Finally, a pedicled strap muscle is fixed to the anterior tracheal suture line with interrupted silk sutures. A closed-suction drain is placed and the incision is closed in layers. Prevention of postoperative hyperextension is ensured by placement of no. 2 vicryl guard suture between the submental crease below the chin and presternal skin at the angle of Louis.
Figure 38.5 Esophageal repair reinforced with pedicled strap muscle.
Figure 38.6 Tracheal repair over a T-tube.
Every effort should be made to extubate the patient in the operating room to prevent excessive positive pressure on the tracheal suture line in the postoperative period. If an air leak is present around the endotracheal tube with the cuff deflated the patient can be safely extubated. Otherwise, a short course of steroids is given and the patient is returned to the operating room in 48 hours for another attempt at extubation. It is often preferable to use a small 5.5 endotracheal tube to lessen irritation of the surrounding tissue.
POSTOPERATIVE MANAGEMENT
Postoperative management after repair of TEF is similar to that for any tracheal resection. The patient is typically observed in an ICU setting for 24 hours postoperatively and maintained on strict NPO status with aspiration precautions. Voice rest is typically not required unless the repair is proximal. Antiemetics are given prophylactically and medications that cause nausea are avoided whenever possible given the potentially disastrous consequences of vomiting. The surgical drain is removed in 3 or 4 days. After 7 days the patient is returned to the operating room for bronchoscopic inspection of the tracheal anastomosis. Endoscopic assessment of the esophageal repair is typically not performed. If the tracheal anastomosis is healing well then the guard sutures may be cut and gentle neck extension is permitted. A barium swallow is performed to confirm the patency of the esophageal repair; if it is normal the patient may initiate a liquid diet with advancement over a period of days.
COMPLICATIONS
Operative mortality following TEF repair has decreased substantially in the last two decades. Most recent series report postoperative mortality under 5%. The operation still carries substantial morbidity. Complications include fistula recurrence, tracheal or esophageal stenosis, recurrent laryngeal nerve injury, pneumonia, wound infection, the need for continued use of tracheal appliance, or failure to progress to an oral diet (Table 38.2). Fistula recurrence can be successfully managed with a second attempt at repair after sufficient time has elapsed for edema and inflammation to subside. Tracheal and esophageal stenosis can be managed with serial dilations and rarely require a second operation. The median hospital stay following repair in one recent study was 14 days.
TABLE 38.2 Morbidity and Mortality Following Repair of TEF
RESULTS
Outcomes following repair of nonmalignant TEF are surprisingly good. A recent review of 38 patients by Muniappan et al. at Massachusetts General Hospital reported that in 94% of patients repair was ultimately successful, 71% were breathing without a tracheal appliance, and 83% were able to resume oral intake. Macchiarini et al. reported on 32 consecutive patients of whom 93% had an excellent or good long-term functional result. Camargo similarly followed 11 patients for a mean time of 32 months and found that 10 reported an excellent functional result. Shen et al. at the Mayo Clinic found that of 35 patients 29 ultimately resumed an oral diet and only one required long-term use of a tracheal appliance.
CONCLUSIONS
Acquired nonmalignant TEF presents a challenge. Optimization of the patient’s overall condition and nutritional status is essential to a good outcome. Mechanical ventilation should be avoided before and after surgery whenever possible. The combination of a carefully positioned tracheostomy tube distal to the fistula and placement of gastrostomy and jejunostomy tube make this possible in the preoperative period. Meticulous attention to technique during the repair gives the patient the greatest chance of successful recovery. When these principles are adhered to 85% of patients can expect a good functional result.
Recommended References and Readings
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Grillo H, ed. Surgery of the Trachea and Bronchi. London: BC Decker Inc; 2004.
Macchiarini P, Verhoye J, Chapelier A, et al. Evaluation and outcome of different surgical techniques for postintubation tracheoesophageal fistula. J Thorac Cardiovasc Surg. 2000;119:268–276.
Mathisen D, Grillo H, Wain J, et al. Management of acquired nonmalignant tracheoesophageal fistula. Ann Thorac Surg. 1990;52:759–765.
Mohebali J, Morse C. Management of acquired esophageal respiratory tract fistula. Cameron J, Cameron A, eds. Current Surgical Therapy. Philadelphia, PA: Elsevier; 2014:767–771.
Muniappan A, Wain J, Wright C, et al. Surgical treatment of nonmalignant tracheoesphogeal fistula: A thirty-five year experience. Ann Thorac Surg. 2013;95;1141–1146.
Shen K, Allen M, Cassivi S, et al. Surgical management of acquired nonmalignant tracheoesophageal and bronchoesophageal fistulae. Ann Thorac Surg. 2010;90:914–919.
Weiser T, Ashiku S, Mathisen D. Tracheal Lesions. Selke F, del Nido P, Swanson S, eds. Sabiston and Spencer: Surgery of the Chest. Philadelphia, PA: Elsevier; 2010:113–127.