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INTRODUCTION

Malignant esophageal fistula occurs infrequently, yet remains one of the most challenging complications encountered in thoracic oncology, with few or no improvements in management or outcomes in the past decade. It may occur in the setting of complicated esophageal cancer, presenting at an advanced stage of disease, or as a complication of treatment. Occasionally, perforation and fistula may complicate the initial presenting diagnostic endoscopy. The fundamental tenets of successful management of any fistula, including relief of distal obstruction, treatment of infection, nutrition, and treatment of underlying malignancy, should be kept in mind but unfortunately are inherent to the disease process and usually overwhelming for patient and clinician. Increased experience with covered self-expanding metal stents (SEMSs) and associated delivery systems has dramatically changed the approach to managing benign esophageal fistulas, particularly involving the tracheobronchial tree. Indeed, nonoperative management of perforations and fistulas associated with benign disease of the esophagus has increasingly proven effective and may include a combination of endoscopic suture, clips, and covered stents or endoluminal vacuum-assisted therapies. Unfortunately, these techniques have not proven appropriate or effective in managing most patients with perforations complicated by malignant disease. Palliation and not cure is the objective in the majority of patients afflicted with this uniformly fatal complication, which most often occurs in the setting of advanced-stage disease. A multidisciplinary approach to accurate and precise diagnosis and treatment is essential.

GENERAL PRINCIPLES: PATIENT EVALUATION AND DIAGNOSIS

Malignant esophageal fistula usually presents with clinical symptoms of recurrent aspiration or less commonly hemoptysis or hematemesis. An esophageal fistula may also be suspected on the basis of transmission of oral flora through the fistula tract and the development of associated infection of the body cavity or organ in communication with the esophagus, such as recurrent pneumonia, empyema, mediastinitis, and abscess. The majority of patients will present with known locally advanced or metastatic tumor and will frequently already be undergoing treatment with chemotherapy and radiation. Alternatively, a previously undiagnosed patient with dysphagia undergoing initial endoscopic evaluation of an esophageal malignancy may present with a perforation and fistula as a complication of the endoscopic procedure. The diagnosis is confirmed with fluoroscopy during administration of dilute barium oral contrast followed by thin-cut computed tomography (CT), which will usually define the precise location and extent of the fistula. A small fistula may be missed on radiographic evaluation, and aspiration of swallowed barium can be confused with a fistula to the respiratory tract, particularly with proximal third esophageal lesions. Flexible esophagoscopy and bronchoscopy often are required for confirmation and anatomic assessment of the suspected fistula and provide additional information needed for treatment planning in addition to the therapeutic benefit of clearing the contaminated airway.

A tracheobronchial–esophageal fistula is often visualized more easily with bronchoscopy rather than esophagoscopy, as the esophageal mucosal folds and tumor sometimes obscure the origin of the fistula. It is also important to visualize any luminal compromise of the airway ...

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