Adult Chest Surgery

Chapter 23. Management of Malignant Esophageal Fistula 

 

Malignant esophageal fistula is a difficult complication of a devastating illness. Its management can be trying for even the most experienced of surgeons. A team approach is necessitated by the demanding and complex nature of the underlying biologic process. The surgeon must have specialized skills for operating on the esophagus, expertise in endoscopy, and cooperation from oncology, pulmonology, gastroenterology, otolaryngology, nutrition, and radiation oncology, among other hospital departments. Locally advanced malignant esophageal fistulas have been described in the upper, middle, and lower esophagus. Fistulas can involve the trachea, bronchi, pleural space, lung, mediastinum, peritoneum, pericardium, and even the great vessels. The organ or space affected by the spread of cancer is integral to devising an effective treatment plan whether the goal is curative resection, rarely an option for these patients, or palliation. While malignant esophageal fistulas can occur anywhere along the course of the esophagus, the form most commonly encountered in thoracic surgical practice is the esophagorespiratory fistula and hence the principal focus of this chapter.

GENERAL PRINCIPLES

Fistulas complicate locally advanced esophageal carcinoma at a rate of approximately 13%.1 In staging patients with esophageal or proximal stomach malignancies, the presence of a malignant fistula is considered T4disease according to the American Joint Cancer Committee (AJCC) Cancer Staging Manual2 because the process invades contiguous structures. Preoperative T4 disease is minimally categorized as stage III disease and usually is deemed unresectable. Without nodal or metastatic involvement (T4N0M0), maximum 5-year survival is 15%,3 but lower survival rates are usually reported. Survival diminishes for the subgroup of patients with esophagorespiratory fistula, and nodal spread compounds the condition, further reducing survival.

Controlling the primary disease process is an important principle of surgical treatment. Long-term survival is a rare possibility for patients with esophageal cancer and is confined to primary tumors that have not transgressed the esophagus. In patients who remain free of nodal or metastatic involvement (N0M0), aggressive chemoradiation may be undertaken, and if the patient has a complete pathologic response, long-term survival is possible. Surgical resection for cure thus is recommended for esophagorespiratory fistulas in patients with N0M0 disease who have a complete pathologic response to neoadjuvant therapy. When the esophagorespiratory fistula is located distally in the respiratory tree, en-bloc pulmonary resection may be considered, and the patient is fully staged at T4N0M0.4 Even this approach is considered controversial because in the setting of microscopic nodal disease, which is probable if the primary disease has led to development of an esophagorespiratory fistula, extensive resection would not be recommended. This is underscored by the fact that in patients in whom preoperative T4N0M0 disease has been fully resected, the cause of mortality is usually distant disease, not local recurrence.

The basic tenet of surgical treatment of esophagorespiratory fistula is soilage control. Even a locally confined cancer can result in high mortality from respiratory embarrassment caused by spillage into the airway. Sepsis from spillage into body cavities truncates survival. There are multiple methods of achieving surgical control: bypass, resection, diversion, covered stent, and chemoradiation.

Supportive therapy to control symptoms, supplement nutrition, and preserve performance status is likewise essential. Dysphagia relief and symptom control improve the patient's performance status and quality of life. It is important for the surgeon to recognize that most patients will not survive this cancer, but their remaining time can be improved with palliative maneuvers.

After spoilage control, attention turns to management of nutrition and opportunistic infections. Infection and nutrition work hand in hand. Poor nutrition leads to immune suppression, poor healing, and rapid decline. Although minimizing spillage will help to control infection, further steps may be needed, including drainage of contained infection, stenting, or even resection and washout. Finally, although relief of dysphagia is often more important to patients than nutrition,5 alimentary access via jejunostomy tube placement may be necessary for survival.

OPTIONS FOR TREATMENT

Many patients with esophagorespiratory fistulas are deemed unresectable based on clinical staging, and palliation becomes the immediate surgical goal. Nodal status in the absence of distant metastases will determine whether a reasonable goal of survival should be pursued. While patients with minimal soilage can be considered for neoadjuvant chemoradiation, the most immediate issue is respiratory compromise. Patients with respiratory embarrassment or sepsis caused by a fistulous process should be managed quickly and aggressively. Preliminary assessment of the patient's performance status and disease stage will define the appropriateness of stenting, diversion, chemoradiation, or resection. Patients in immediate danger from respiratory soilage should undergo endoscopy for a quick assessment of the nature of the fistula concomitant with immediate treatment. In this circumstance, protective stenting can be performed with expediency as the first option.

Stenting with self-expanding metallic stents is probably the most common treatment for esophagorespiratory fistula. Ease of use, expediency of delivery, and efficacy are all attractive features of this modality. Several trials have documented closure rates in the range of 75–100% with resumption of a nearly full oral diet.6 Stenting can be performed under conscious sedation with a mortality rate significantly lower than that of enteric bypass, returning the patient to his or her life as soon as possible.7

The site of the fistula/tumor also influences therapy. If the process is located in the cervical esophagus, stenting may be less desirable. Cervical stents can add to the patient's discomfort and exacerbate dysphagia. Patients often report an uncomfortable sensation "of a foreign body." Nonetheless, stenting still can be a viable and reasonable option. Cervical esophageal cancer is also predominantly squamous cell carcinoma in origin and responds well to chemoradiotherapy, which can be initiated independently or in combination with stenting.

Proximal lesions without distant disease and nodal spread may be suitable for pharyngolaryngoesophagectomy. Much of the management of cervical esophageal cancer with contiguous organ involvement is discussed in the otolaryngology literature.8 These data support the use of neoadjuvant chemoradiation because it theoretically reduces the prevalence of micrometastases.9

Stenting is also difficult at the gastroesophageal junction, where stent migration and reflux esophagitis can cause difficult problems. Some stents, such as the Esophageal Z-Stent with Dua Anti-Reflux Valve (Cook Medical, Inc., Bloomington, IN), are designed with one-way valves and clever windsock conformations devised to combat reflux.

Palliative chemoradiotherapy has been shown to be both safe and effective for malignant esophageal fistulas, whether alone or in conjunction with other modalities.10 However, it takes time for the treatment to take effect, and the treatment is also associated with certain risks. Chemoradiotherapy is effective for closing off small fistulas, but large fistulas may require additional intervention. Delayed spillage also may occur when small fistulas occlude secondary to acute edema only to recanalize when the swelling recedes. Despite these associated risks, palliative chemoradiation is increasing in popularity,11 whereas stent use, which often requires repeat instrumentation, appears to be declining.12

Bypass and diversion may be a useful option in patients with esophagorespiratory fistulas who have reasonable performance status and are without metastatic and/or bulky nodal disease. These patients may survive long enough to benefit from this operative approach. Although temporarily debilitating, bypass and diversion can relieve the patient of dysphagia and aspiration, which cause considerable debilitation and discomfort.

Neoadjuvant chemoradiation followed by resection is a less common approach that applies only to patients with excellent performance status, who have been fully staged as T4N0M0, and have a fistula that involves a resectable structure. All the aforementioned conditions make this cohort of patients exceedingly small. Even then, these procedures are best performed in select centers with adequate experience.13

Middle and lower thoracoesophageal fistulas frequently involve the distal airway but rarely involve lung parenchyma or cause free pleural perforation. A complete preoperative workup including CT scan, esophagoscopy, and bronchoscopy is essential. In considering the potential fistula endpoints, one must account for all the various structures that abut the esophagus. Pericardium, peritoneum, and aorta are potential endpoints, however rare. Pericardial fistula is a devastating but exceedingly rare complication. It is a profoundly symptomatic event, with patients often displaying signs of heart failure, arrhythmia, pericarditis, sepsis, and pneumopericardium.14 Although potentially diagnosed with chest radiograph and physical examination (e.g., Hamman's crunch or the pulsatile sound of pneumomediastinum), CT scan is recommended, followed by urgent/emergent operative intervention. Pericardial window, washout, and drainage are essential. Further intervention is determined based on fistula height and the condition of the patient. If the patient presents in critical condition, an esophagectomy should be avoided. The operative mortality for emergency esophagectomy owing to perforated fistulous cancer has been reported to be as high as 50%. Stenting in conjunction with a pericardial window is an attractive option. Cases are highly individual, and the treatment must be tailored appropriately to the situation.

Abdominal esophageal cancers can "fistulize" to the peritoneum. Patients with this entity often present with an acute abdomen. This diagnosis carries a dismal prognosis. If resection of perforated gastroesophageal junction cancer is pursued, the intraoperative mortality alone approaches 50%.

A malignant fistula may "evolve" during treatment and occasionally perforate. Causes include tumor necrosis, primary invasion into contiguous structures, and iatrogenic injury related to instrumentation or radiation. Perforation is always associated with a poor prognosis. Emergent resection carries tremendous risk but may be the only choice. Efficient and minimal interventions are essential. In this setting, the goals are controlling spillage and returning the patient to the ICU because an extensive R0 resection would be associated with high morbidity and poor survival.

Fistulization to the aorta is usually lethal. This process can present as a "sentinel" or "herald" bleed and provides a limited window of opportunity during which intervention can be attempted. Treatment options include endoluminal aortic stenting, axillofemoral bypass with ligation of the fistulous area, or even replacement of the fistulous segment with homograft followed by intervening flap. All these options depend on the capabilities of the institution and the condition of the patient. Time is not a luxury in these cases, and often nothing can be done to prevent exsanguination.

Performance status is important when selecting treatment. Many patients with malignant esophageal fistulas cannot tolerate major operative procedures. For these patients, quick and efficient palliation should be the goal.

PREOPERATIVE ASSESSMENT

In the rare patient who is being seriously considered for a "curative" or major operative approach, the preoperative assessment begins with esophagoscopy followed by CT scan of the chest and abdomen to assess for intrathoracic and intraabdominal extent of disease. Endoscopic esophageal ultrasound to evaluate T and N stages, combined with fine-needle aspiration for accurate tissue diagnosis, also should be performed. The preoperative assessment is more limited in patients who are clearly unresectable. For instance, a large esophagorespiratory fistula of the main trachea involving multiple rings presenting in a patient coughing up gastrointestinal contents would not call for esophageal ultrasound. Bulky fistulizing tumors of this type often can be diagnosed primarily with endoscopy and biopsy, followed by palliative stenting of the esophagus and/or airway to control soilage. PET scan, esophageal ultrasound, and barium swallow are unnecessary studies in the setting of acute airway soilage from esophagorespiratory fistula. These tests are reserved for patients who have an unclear extent of disease. For instance, a patient who presents with a cough secondary to a tumor of the esophagus that impinges on but does not clearly involve the trachea requires further investigation. This is an ideal patient for esophageal ultrasound, which can evaluate with high specificity the extent of local invasion of the primary tumor.

TECHNIQUES

Stenting

Techniques for esophageal and tracheal stenting are presented in Chaps. 22 and 47, respectively. One must be prepared to perform either or both when evaluating a patient with a malignant esophageal fistula. The specific techniques are not covered here; rather, special circumstances and important caveats are reviewed. Once the stent has been positioned, closure of the malignant esophageal fistula can be confirmed with a series of barium swallows of progressive thickness. After stent placement, bulky foods should be avoided for at least 1 week because self-expanding metallic stents will continue to expand and incorporate into the esophageal wall over this interval.6 Overall experience in stent deployment is an important skill set for surgeons caring for these patients.

High Fistula

Esophagorespiratory fistula of the cervical esophagus presents a unique problem. Stenting, while still an option, can be less successful for palliation because of foreign-body sensation or luminal airway compromise, and attention may be turned to resection. Patients with poor performance status who require quick intervention may benefit most from stenting.

In selected patients, pharyngolaryngoesophagectomy is considered a viable option for palliation and rarely may provide cure. This is a technically difficult and extensive surgery. Broad preoperative and intraoperative antibiotic coverage is basic to extensive head and neck resection with alimentary reconstitution. Surgical resection may require the combination of standard esophagectomy with laryngectomy, mediastinal tracheostomy, hemithyroidectomy, and challenging restoration of alimentary continuity. Preoperative bowel preparation is essential in the most basic of esophageal operations. Three-hole esophagectomy is useful here with the addition of manubriectomy. The resection of the manubrium, clavicular heads, and chondral heads of the first and second ribs can provide the amount of skin needed to meet the edges of a mediastinal tracheostomy. Pectoralis flaps can be advanced over the bare edges of bone to decrease sharp pressure against and provide separation between the airway, the alimentary tract, and vascular structures. The laryngectomy and hemithyroidectomy are performed leaving the bare end of the trachea. This resection should be performed in conjunction with an experienced laryngeal surgeon. The trachea should be dissected out and pulled anteriorly and laterally to the right side of the innominate artery. This adds length to the tracheal stump and reduces pressure between the artery and airway, helping to prevent tracheoinnominate fistula. An in-depth description of mediastinal tracheostomy is provided in Chapter 57.

Restoration of oral-intestinal continuity is challenging and controversial. While it is reasonable to perform a direct gastric tube–pharyngeal anastomosis, there is a high rate of anastomotic leak. The stomach, if it reaches, is at maximum length and will produce some tension on the anastomosis. Because the anastomosis is in the neck, a leak is easily dealt with via drainage and usually will resolve promptly. Using a free jejunal graft is another option. Microvascular anastomosis of a free jejunal graft to the external carotid artery and branches of the jugular vein is the most common approach to this problem. Transillumination of the mesentery of the jejunum should be performed to isolate an appropriate and convenient vascular arcade. The identified jejunal segment should be divided and the proximal limb marked. Next, the recipient vessels should be prepared for anastomosis. The arcade vessels then are divided. The vein-to-vein anastomosis should be performed followed by the arterial anastomosis. Standard techniques for microvascular anastomoses are followed, including the tension-free technique. If needed, saphenous vein extension may be used to relieve tension. Finally, isoperistaltic jejunal anastomosis is performed proximally with the oropharynx and distally with the gastric tube. The proximal anastomosis should be performed in a single layer of interrupted sutures. Often there is a size mismatch between the oropharynx and jejunum. This is overcome by spatulating the jejunum along its antimesenteric border proximally until the two lumens are identical (Fig. 23-1). The distal anastomosis may be stapled or hand sewn.

Figure 23-1.

 

Restoration of oral-intestinal continuity using a jejunal free flap in a patient with high (cervical) esophageal respiratory fistula. To account for the difference in diameter between the oropharynx and jejunum, the jejunum is spatulated along the antimesenteric border until the lumen of the oropharynx and jejunum are of identical diameter and can be anastomosed.

 

The colonic conduit is supported by many surgeons for cervical interposition for multiple reasons. The procedure is simpler to perform than a free flap. The colon more easily matches with the oropharyngeal size and usually can reach without tension, therefore decreasing the leak rate. The complaint of positional gastric secretion reflux is not an issue after colonic interposition. For these reasons, colonic interposition is considered by some surgeons as the conduit of choice after pharyngolaryngoesophagectomy. Other techniques, such as the forearm free flap or thigh free flap formed into an epithelial-lined tube, are well described. An antebrachial free flap is a reliable source for epithelialized vascular pedicle and can be harvested by a separate surgical team simultaneously. Consult or assistance from plastic surgical colleagues with specific experience in free flaps, as well as split-thickness skin grafts, is useful for this stage of the operation. Before going to surgery, a standard Allen test should be performed to confirm the integrity of the palmar arch. A tourniquet and circumferential preparation should be applied to the nondominant arm. A rectangular area on the volar surface is marked off, approximately 5 cm transversely and 10 cm longitudinally (adjusted for need). Space allotted between the wrist crease and the graft area makes the split-thickness skin graft more reliable and yields a better cosmetic result. Dissection is accomplished down to the muscular capsule of the mobile wad and volar compartments on either side longitudinally (Fig. 23-2). The distal radial vessels are ligated and dissected, along with the graft, from a distal to proximal approach, with care to preserve the radial nerve branches. Before detaching, the flap is rolled to form a tube longitudinally and sewn along its edge in a double layer. The internal epithelial surface is run with 3-0 or 4-0 PDS and the outer layer with 3-0 Vicryl. Similar to the jejunal free flap, the proximal radial artery and cephalic vein are not divided until the recipient vessels are prepared. As with jejunum, this conduit may provide continuity between the pharynx and either colonic or gastric replacement.

Figure 23-2.

 

An antebrachial free flap is a reliable source of epithelialized vascular pedicle. Like jejunum, this conduit may provide continuity between the pharynx and either colonic or gastric replacement. Dissection is made down to the muscular capsule of the mobile wad and volar compartments on either side longitudinally. The distal radial vessels are ligated and dissected, along with the graft, from distal to proximal, with care exercised to preserve the radial nerve branches. Before detaching, the flap is rolled to form a tube longitudinally and sewn along its edge in a double layer.

Esophagorespiratory Fistula of the Thorax

Stenting is a valuable option. Self-expanding covered stents are perhaps the most frequently used method of palliation for esophagorespiratory fistula of the thorax. Bulky cancer that fistulizes to the trachea often will respond to stenting of the esophagus alone. This usually will stop the soilage and relieve the dysphagia. A covered stent is mandatory. Occasionally, both airway and esophageal stents must be employed to quell external compression of the trachea by the stented bulky esophageal tumor. Many kinds of esophageal stents are used today, including simple plastic tubes (with distal flange and proximal funnel), covered self-expanding metal stents, the Alimaxx-E (Alveolus, Inc., Charlotte, NC), the Wallstent (Boston Scientific Corporation, Natick, MA), the Z-Stent (Cook Medical, Inc., Bloomington, IN), the Ultraflex or Polyflex (Boston Scientific, Natick, MA), and many more.

Tracheobronchial stents are discussed in detail in Chapter 47. As stated previously, these stents may be needed if the external bulk of the tumor compromises the airway lumen. Esophageal stents are often all that one needs, but the surgeon needs to be prepared for double stenting of both airway and esophagus.

Another option is exclusion and bypass of the fistulous portion of the esophagus. This would seem to be an ideal solution to this problem because alimentary continuity and control of soilage are achieved simultaneously. However, this is only viable if patient performance status is very high. A patent fistula may be left in place while retrosternal bypass restores continuity. This approach necessitates placing the patient in the supine position. Neck and upper abdominal incisions are used to exclude the esophagus and tunnel the conduit substernally. The fistula can be left in position without risk of abscess because the contents of the wide-open fistula simply drain into the trachea, and because it has been excluded, the mucus produced by the esophageal remnant is clinically insignificant. Mucocele and abscess could be potential late complications but are obviated by short patient survival. Continuity is achieved with a tubularized gastric pull-up through the anterior mediastinum (Fig. 23-3). Blunt dissection is performed from the sternal notch and subxiphoid space in the anterior mediastinum. Digital and "sponge stick" dissections are useful. Once a generous tunnel is complete, the pull-through can proceed. In most cases it is best as a prophylactic measure to resect the head of one or both clavicles, part of the manubrium, and potentially the proximal anterior first ribs to prevent mechanical obstruction of the conduit owing to a small thoracic inlet. If there is any question of a small aperture, it is better to be conservative. Unilateral clavicular head resection may be sufficient.

Figure 23-3.

 

Malignant esophagorespiratory fistula of the thorax treated surgically by exclusion of the fistulous portion of the esophagus and retrosternal bypass. The esophagus is excluded through neck and abdominal incisions. The fistulous portion of the esophagus is left in place because its contents drain into the trachea. Retrosternal bypass with a tubularized gastric pull-up through the anterior mediastinum restores continuity of the alimentary canal.

 

Occasionally, this situation may arise in a patient with a frozen anterior mediastinum consequent to a previous operation, such as coronary artery bypass grafting. In this situation, antesternal or subcutaneous bypass is a viable option. Antesternal bypass is performed in the same fashion as retrosternal bypass with the exception that a subcutaneous tunnel is used. Although the distance one needs to traverse is several centimeters more, the traditional gastric conduit should suffice (Fig. 23-4). Both retrosternal and antesternal bypass can be successful palliative procedures for malignant esophagorespiratory fistula.

Figure 23-4.

 

Exclusion of malignant esophagorespiratory fistula of the thorax and sub- or antesternal bypass. Antesternal bypass is performed in the same fashion as retrosternal bypass except that a subcutaneous tunnel is used.

In rare circumstances, esophagorespiratory fistula can terminate distally in the pulmonary parenchyma, posing a difficult problem. Chronic pneumonia will not clear as easily as in the case of an excluded fistula to a more proximal airway. Esophageal stenting in conjunction with aggressive antibiotic therapy can be attempted. Chemoradiotherapy may cause small fistulas to close and allow for simple esophageal resection for palliation.4Pulmonary wedge resection in conjunction with esophagectomy for bypass may be used in patients who have excellent performance status. It has been reported that a T4N0M0 esophageal cancer that has fistulized to the pulmonary parenchyma may be approached safely with an esophagectomy combined with anatomic pulmonary resection. However, survival improvements in these limited studies are not powered by significant numbers and most certainly include selection bias.13

Esophagopleural Fistula

Esophagopleural fistula is the term used for a perforated thoracic esophageal cancer. The management of this entity depends on the condition of the patient and the containment of soilage. Patients with free pleural perforations may present with full-blown sepsis. These patients require antibiotics, damage control, and resuscitation. Mortality in perforated cancer is exceedingly high. Therefore, minimal resection, vigorous lavage, and adequate drainage are primary points. Restoration of alimentary continuity can be performed if the soilage is minimal and the patient stable, but bypass probably should be reserved for a separate time. If the perforation is contained (i.e., not freely mobile within the pleura), tube drainage combined with esophageal stenting is more desirable than open intervention. Perforation into a loculated space often can be drained percutaneously with CT guidance.

Esophagoperitoneal Fistula

Esophagoperitoneal fistula is synonymous with malignant intraabdominal perforation. Once again, this is a catastrophic event associated with an already difficult process. In most cases, resection will be too physiologically demanding for the patient who presents in septic shock, and therefore, diversion, washout, and drainage are used to temporize the patient. The fistula can be diverted by performing cervical esophagostomy (Fig. 23-5). This procedure is similar to the cervical phase of a transhiatal esophagectomy. The esophagostomy should be brought out through a subcutaneous tunnel below the level of the clavicle, if possible. In this position, the ostomy bag will affix to the patient with a better seal, is easier to manage, and yields a better cosmetic result. The abdominal fistula can be washed out and simply drained, if necessary. If the patient's performance status and disease state are amenable, a second-stage resection of the fistula and perforation with combined substernal bypass is an option. However, most patients will not be able to tolerate the physiologic insult associated with this extensive procedure.

Figure 23-5.

 

The term esophagoperitoneal fistula is synonymous with a malignant intraabdominal perforation. Most patients are unable to tolerate a curative resection, and therefore, diversion, washout, and drainage can be used to temporize the patient. A. The procedure for performing the cervical esophagostomy. B. The esophagostomy should be brought out through a subcutaneous tunnel below the level of the clavicle, if possible.

SPECIAL CIRCUMSTANCES

Esophagopericardial Fistula

Esophagopericardial fistula is one of the rarest of fistulas. Antibiotics, drainage of the pericardium, washout, and stenting can be combined in the acute phase to deal with this catastrophic event. If the patient has excellent performance status and is without obvious metastases or bulky nodal disease, resection including the pericardium for palliation may be considered, but again, a large operation should be avoided in the acutely ill and septic patient.

Esophagoarterial Fistula

Survival with fistula of the esophagus to the aorta depends on early recognition in a patient presenting with a "herald" bleed. Such patients should be prepared for operation immediately. If possible, the patient should be prepped in the operating room for endovascular stenting of the aorta. This is potentially the quickest and least invasive method of controlling the hemorrhage. Unfortunately, this maneuver effectively creates an infected graft. Individual reports in the literature have shown that antibiotic therapy can be given continuously in conjunction with endovascular grafting as singular therapy.

Other options include bilateral axillofemoral bypass combined with proximal and distal ligation. The difficulty here is catastrophic hemorrhage while performing axillofemoral bypass in preparation for aortic ligation. One can use a transbrachial aortic occlusion balloon without insufflation in anticipation of hemorrhage, just as one would use an aortic cross-clamp. If so, the balloon should be placed in the operating room under fluoroscopy as the first priority and may slow a major aortoenteric hemorrhage sufficient for direct open intervention. In these situations, the surgeon should possess advanced endovascular skills or work in conjunction with an appropriate interventionalist.

POSTOPERATIVE CARE

Postoperative management of the malignant esophageal fistula patient is based on the patient's disease stage, site of the fistula, and subsequent procedure. Immediate postoperative care for most patients undergoing major resection and bypass requires ICU admission and parenteral nutrition while awaiting return of bowel function. A barium swallow study on postoperative days 5–7 is an acceptable precursor to feeding a gastric or colonic conduit at our institution.

Patients undergoing free-flap procedures are treated with steroids for approximately 72 hours postoperatively to decrease autograft edema and ischemia-reperfusion injuries in the immediate postoperative interval. Aspirin should be given per rectum to prevent vascular thrombosis. Swallow studies should be performed 8–14 days postoperatively before instituting feeding. A more conservative approach to testing free grafts is taken because minor leaks are far less well tolerated in these grafts.

Heparin subcutaneously three times per day should be given to all these patients. Perioperative antibiotics should be given according to each hospital's formulary and continued for 2 days postoperatively. Our institution typically will use cefazolin every 8 hours for six doses unless obvious allergy or renal issues force a change.

Every surgeon, general or thoracic, has a slightly different view on when and how to begin feeding after surgery. We usually begin feeding down the jejunostomy tube on postoperative day 3, with trophic supplementation to stimulate mucosal proliferation, which is low in jejunostomy patients, if the patient does not have a free jejunal flap taken from distal bowel. Tube feeding is ramped up with return of bowel function or in accordance with surgeon preference. In the case of free perforation into the pleural, peritoneal, or pericardial space, broad-spectrum antibiotics must be given, and strong consideration should be given to antifungal agents. Broad-spectrum antibiotics in the face of critical illness, often combined with total parenteral nutrition, is reason enough for many critical care clinicians to begin antifungal therapy empirically. In the case of endovascular stenting of an esophagoarterial fistula, lifelong antibiotic therapy may be a reasonable approach.

COMPLICATIONS

Because stenting is the most common method of treating esophagorespiratory fistulas, poststent complications are a primary concern. Determining the clinical significance of the potential complication is key. A small radiologic leak of contrast material into the airway that persists after an esophageal stent is placed is not necessarily significant if the patient is asymptomatic. If the patient has a resulting cough that is intolerable, however, the problem is significant. In this case, overlapping esophageal stents may suffice, or it may be necessary to stent the airway side to provide relief.

High stents can cause the sensation of a foreign body, a side effect that some patients find quite tolerable. Others are debilitated by the feeling and report that it interferes with their ability to rest. The decision to remove the stent must be weighed against the morbidity of a fistula bypass procedure or pharyngolaryngoesophagectomy. For some patients, stenting may be the only option.

If a bulky cancer is found to encroach on the airway after the esophageal stent has been placed, it must be removed immediately. After airway patency is ensured, alternative stenting of the airway side may be undertaken. In the case of double stenting, the tracheobronchial stent should be placed first.

As with all forms of esophageal cancer surgery, pneumonia is a primary concern. Pneumonia can be an unavoidable or even preexisting complication of malignant esophagorespiratory fistula. Aggressive postoperative pulmonary toilet is essential. Early extubation and adequate pain control are the basic tenets of care for patients with esophagorespiratory fistulas. However, one must be certain before extubating the patient with esophageal bypass that he or she can breathe spontaneously and clear secretions because accidental replacement of an endotracheal tube into the esophagus can dehisce the cervical anastomosis.

Anastomotic leaks in the context of free flaps can have dire outcomes. Prevention is key, and measures to avoid this complication center on maintaining graft viability. Free-flap vascular thrombosis can be caused by infection or hematoma. Perigraft drains should be left postoperatively and monitored vigilantly. Steroids are administered to reduce swelling and reperfusion injury in the flap itself. Aspirin given by rectal suppository is also an important adjunct when working to prevent thrombosis. Subcutaneous heparin, 5000 U three times daily, can help to decrease thrombosis rates. The head and neck literature reports use of indwelling Doppler catheters placed adjacent to the vascular pedicle, which can be monitored and simply pulled after 48 hours. These may be particularly helpful in head and neck procedures, where the graft may be difficult to visualize.

Esophagostomy is associated with a unique set of complications. As with any neck dissection, hematoma and recurrent laryngeal nerve injury are concerns. With esophagostomy, the distal end of the cervical esophagus can develop some element of stenosis. Installing a tube, which is passed nasally through the esophagus out of the ostomy, for the first few days after surgery combats this issue. Additionally, if a second-stage alimentary continuity operation is undertaken, the distal esophagostomy should be debrided to decrease the likelihood of anastomotic leak or ischemic stricture.

SUMMARY

Development of a malignant esophageal fistula is a catastrophic event with devastating consequences for the patient. Immediate efforts should focus on palliation, and the remoteness of curative resection should make staging a very short and efficient effort. Procedures that combine staging with palliative treatments, such as endoscopy and stenting to maximize quality of life, should be planned whenever feasible. Most patients with malignant esophageal fistulas will undergo some form of stenting, but few undergo resection because of the prohibitive risks. For the rare patient who is considered suitable for elective resection or bypass, the surgeon must be prepared to undertake creative and infrequently performed surgeries that are tailored to the individual patient. The occasional presentation of a perforated malignant fistula will lead the surgeon to the operative theater for urgent management to quell the hemorrhage, minimize soilage, resuscitate, and drain.

CASE HISTORY

A 52-year-old woman with progressive dysphagia presented to her primary care physician. Laboratory samples were drawn but found to be grossly normal, and the patient was referred to the gastroenterology service at our hospital. A CT scan revealed a bulky mass of the high thoracic esophagus and shotty lymphadenopathy along the esophagus and stomach. Esophagoscopy and biopsy were performed and revealed a large tracheoesophageal fistula through which the esophagoscope easily passed. The pathology report was positive for squamous cell carcinoma of the esophagus. The patient was referred to the thoracic surgery service for potential resection or bypass. After evaluation in the office, the patient was admitted and taken to the OR for esophageal stenting. Esophagoscopy, bronchoscopy, and dual stenting were performed to provide airway stability and relieve dysphagia. The patient was extubated and monitored postoperatively in the ICU. After the patient was stabilized for 2 days, she was returned to the OR for elective jejunostomy. The patient recovered well enough to be discharged home under hospice care.

EDITOR'S COMMENT

A serious complication of esophageal cancer, malignant fistula is extremely hard to manage. The best options include palliation with stenting or, when absolutely necessary, local muscle flap rotation. Resection and replacement is a hazardous procedure in these patients with poor nutrition and limited survival and should be approached, I believe with some trepidation.

–MJK

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