Tex Heart Inst J. 2006; 33(1): 60–62.

Late Manifestation of a Large Congenital Tracheoesophageal Fistula in an Adult

Pankaj Saxena, MCh and Robert Tam, FRACS

Department of Cardiothoracic Surgery, The Prince Charles Hospital, Brisbane, QLD, 4032 Australia


Congenital tracheoesophageal fistula in an adult is a rare condition. We describe the clinical presentation in a young woman after video-assisted thoracoscopic pleurodesis for spontaneous pneumothorax. She was found to have a very wide and short fistula in her neck. The tracheoesophageal defect was closed by a trap-door flap, using the posterior wall of the trachea. The patient made an uncomplicated recovery.

Keywords: Tracheoesophageal fistula/ congenital/surgery

Congenital tracheoesophageal fistula in an adult is a rare condition. We describe the clinical presentation in a young woman after video-assisted thoracoscopic pleurodesis for spontaneous pneumothorax.

>Case Report 
Case Report

In December 2002, a 22-year-old woman presented with chest pain of 4 days' duration. She was found to have a right spontaneous tension pneumothorax. The patient was managed initially with a pleural drain. She continued to have air leakage for 6 days. She underwent video-assisted thoracoscopic pleurodesis. Subpleural blebs were found and were resected with an endoscopic stapler (Ethicon, Inc.; Somerville, NJ), and abrasive pleurodesis was performed. The patient appeared to make an uncomplicated recovery; but 3 weeks later, she started developing dyspepsia, bronchospasm, and a choking sensation upon the ingestion of liquids, and she had an episode of aspiration pneumonia.

Bronchoscopy, esophagoscopy, and magnetic resonance imaging (MRI) confirmed the presence of a tracheoesophageal fistula (Figs. 1 and 2). She was managed initially with a nasogastric tube for decompression of the stomach, and later she underwent percutaneous gastrostomy for the insertion of a feeding tube. Intravenous antibiotic agents were used for treatment of the aspiration pneumonia.

The patient was scheduled for semi-elective repair. General anesthesia was instituted with orotracheal intubation. The neck was placed in a hyperextended position, and a transverse cervical incision was made. The strap muscles were retracted. The platysma and the isthmus of the thyroid gland were divided. Dissection was performed to expose the cervical trachea. At this stage, a fiberoptic bronchoscope was introduced, and the site of the tracheoesophageal fistula was located. Aided by the bronchoscope, we introduced a 16-gauge needle through the wound to locate the site of the fistula. The opening of the fistula was 1 cm. A transtracheal repair was planned. A flap of posterior wall of the trachea adjacent to the fistulous opening was mobilized and was used to close the defect (Fig. 3). A nasogastric tube was placed in the esophagus to facilitate the 2-layer repair, which was accomplished with 3-0 Vicryl suture (Ethicon) for the inner layer and 3-0 silk suture for the outer layer. The inner layer was inverted to close the mucosal defect. The outer layer included the muscular layer of the esophagus and incorporated the tracheal flap from the posterior wall and the wall of the fistula. In order to avoid injury, we made no particular attempt to expose the recurrent laryngeal nerves. Ventilation was resumed at this stage by direct intubation of the trachea distally. The trachea was repaired in an end-to-end fashion using 3-0 Vicryl suture. The posterior layer was sutured in a continuous fashion, and interrupted sutures were used to complete the suture line anteriorly. The endotracheal tube was then repositioned through the oral cavity and was placed beyond the anastomotic site. A pedicled flap of sternohyoid muscle was interposed between the 2 suture lines to separate them. The patient made satisfactory postoperative recovery.

At histopathologic examination, mucosal membrane and, more specifically, muscularis mucosae were found, which confirmed the diagnosis of congenital fistula. There was no evidence of inflammation or malignancy. A barium swallow, done postoperatively, showed the absence of any tracheoesophageal communication. Follow-up at 2 years confirmed a satisfactory clinical condition.


Congenital tracheoesophageal fistula without esopha-geal atresia is a rare condition in adult patients. A strong clinical suspicion is needed to establish the diagnosis. The presentation is in the form of frequent respiratory infections, coughing episodes when eating or drinking, paroxysmal cough, and hemoptysis. The diagnosis is sometimes made at the time of endotracheal intubation for some other surgical procedure. The diagnosis is established with bronchoscopy, esophagoscopy, and barium swallow. Surgery is indicated for the control of symptoms and to prevent and treat associated pulmonary sepsis.

Various explanations are offered for late manifestation of this congenital anomaly ---oblique course of the fistula, the presence of a membrane blocking the lumen of the fistula, the presence of a valve mechanism, and spasm of the fistulous tract.1,2 In our patient, a membrane-like structure was seen to shield, in part, the opening of the fistula. Probably more than 1 mechanism was responsible for the delayed presentation in our patient. Congenital fistulae are relatively long and narrow in their course, unlike the fistula in our patient.3 Nonmalignant tracheoesophageal fistulae associated with the use of a cuffed tracheostomy and endotracheal tubes are also different from these fistulae. Grillo has laid out the principles of surgical management of the acquired fistulae.4,5 The surgical management of tracheoesophageal fistulae has been ligation or simple division and repair of the trachea and esophagus. Pulmonary resection may be needed when chronic sepsis has destroyed a segment or lobe of lung.

A case report from the Mayo Clinic6 described a similar transtracheal method of repair for a short, wide tracheoesophageal fistula in an adult patient. In that case, there was associated achalasia of the esophagus, which was a confounding factor. The diagnosis of fistula could be made only after esophagomyotomy. The fistula-bearing portion of the trachea was resected. The esophageal defect was closed directly, and the 2 ends of the trachea were anastomosed end-to-end with nonabsorbable suture.

We used an unconventional technique for the management of an entity that can be difficult to diagnose in an adult. For the repair of a large fistula, closure of the defect in the tracheal aspect may require a patch. The present method, with division of the trachea, provides excellent exposure of the fistula. This technique is justified in a patient like ours, who has a wide, short fistulous communication. It enables secure closure of the esophageal defect with autologous tissue. We think that the use of a flap of posterior wall of the trachea for repairing the defect may provide a better approach, due to the tissue's vascularity and buttressing effect. Also, this technique helps to avoid loss of vascularity by requiring less mobilization of the trachea. Another advantage is that the esophageal and tracheal suture lines lie at different levels, which reduces the chance of recurrence.


Address for reprints: Pankaj Saxena, MCh, DNB, Senior Registrar, Department of Cardiothoracic Surgery, Sir Charles Gairdner Hospital, Hospital Avenue, Nedlands, WA, 6009 Australia

E-mail: drpankajsaxena@hotmail.com

Grant DM, Thompson GE. Diagnosis of congenital tracheoesophageal fistula in the adolescent and adult. Anesthesiology 1978;49:139 --40. [PubMed].
Enoksen A, Lovaas J, Haavik PE. Congenital tracheo-oesophageal fistula in the adult. Report of a case and review of the literature. Scand J Thorac Cardiovasc Surg 1979;13: 173 --6. [PubMed].
Blackburn B, Amoury RA. Congenital esophago-pulmonary fistulas without esophageal atresia: an analysis of 260 fistulas in infants, children and adults. Rev Surg 1966;23: 153 --75. [PubMed].
Grillo HC, Moncure AC, McEnany MT. Repair of inflammatory tracheoesophageal fistula. Ann Thorac Surg 1976; 22:112 --9. [PubMed].
Mathisen DJ, Grillo HC, Wain JC, Hilgenberg AD. Management of acquired nonmalignant tracheoesophageal fistula. Ann Thorac Surg 1991;52:759 --65. [PubMed].
Olivet RT, Payne WS. Congenital H-type tracheoesophageal fistula complicated by achalasia in an adult: report of a case. Mayo Clinic Proc 1975;50:464 --8.
Figures and Tables
figure 15FF1
Fig. 1 Bronchoscopic view of large tracheoesophageal fistula.
figure 15FF2
Fig. 2 A magnetic resonance imaging scan outlines the fistula.
figure 15FF3
Fig. 3 Diagram of the tracheoesophageal fistula as seen during repair. The trachea has been divided, and the opening of the fistula, the flap of posterior tracheal wall, and the anterior wall of the esophagus are visible.