Cervical approach for repair of congenital tracheo-esophageal fistula presenting in an adult.KV Albuquerque, RK Deshpande, PB Desai
Tata Memorial Hospital, Parel, Bombay, Maharashtra.
Congenital tracheo-esophageal fistula without esophageal atresia was diagnosed on barium swallow radiography and esophagoscopy findings in an adult patient presenting with difficult in swallowing. The fistulous opening seen on the anterior wall of the esophagus led to the trachea. On bronchoscopy, the tracheal opening was located posteriorly 5 cm below the vocal cords. Successful repair was undertaken via a left cervical approach. The anatomical and developmental basis for the preferred approach is discussed with a brief literature review on the subject.
Keywords: Barium Sulfate, diagnostic use,Bronchoscopy, Case Report, Deglutition Disorders, etiology,Esophagoscopy, Human, Male, Middle Age, Tracheoesophageal Fistula, congenital,diagnosis,epidemiology,surgery,
The H-type of congenital tracheo-oesophageal (TE) fistula, or TE fistula without associated oesophageal atresia is an extremely rare anomaly. While the majority of these TE fistulas are diagnosed in infancy, a few may be identified only in adulthood. This article describes H-type fistula in an adult together with a method for the surgical management of adult TE fistula.
A 48-year-old male presented to the Thoracic Service with a history of difficulty in swallowing and an outside barium swallow with a diagnosis of cancer of the upper third oesophagus. Detailed questioning revealed a history of coughing and choking on taking food, more with liquids than solids for a period of one year.
Clinical examination revealed an averagely built and nourished man. The abdominal examination was normal and there was no cervical lymphadenopathy. The chest showed find crepitations over both lung bases. A peripheral blood count revealed a mild polymorphonuclear leukocytosis, the rest of the blood chemistry was normal. A pulmonary function study and chest x-ray showed no abnormality.
A barium swallow done initially showed a mucosal irregularity in the upper third oesophagus, which on endoscopy appeared to be due to a polyp on the anterior wall at 20 cms. This was biopsied. The rest of the oesophagus was normal. A repeat oesophagoscopy demonstrated a fistulous opening on the anterior wall at 20 cm leading to the trachea. This was confirmed the tracheal opening was located 5 cm below the vocal cords posteriorly to the right. The fistula was seen to be lined by mucosa.
In the absence of any etiological factor and because of the long history with the presence of mucosa lining the fistula, a diagnosis of congenital W type TE fistula was made. As the fistula was located at the level of the 2nd thoracic vertebra [Figure - 1] it was decided to approach the fistula for transection through the left neck.
Surgical Procedure. An incision was made along the anterior border of the left sternomastoid muscle and the tracheooesophageal groove exposed carefully protecting the thyroid, recurrent laryngeal nerve and carotid vessels.
The fistula was identified low in the neck at the level of the manubrium connecting the right postero-lateral wall of the trachea with oesophagus. It was 1.5 cm in diameter and extremely short, covered by thick fibrous tissue. It was divided flush with the oesophagus to aid secure closure of the tracheal end. The oesophageal opening was then repaired in two layers. The divided omohyoid muscle was interposed in between.
The patient had an uneventful recovery and now eats a normal diet without difficulty. He was asymptomatic when last seen six months after the procedure.
Congenital oesophago-respiratory fistula is a rare entity, generally seen in combination with oesophageal atresia. The ‘H’ type of TE fistula, is even rare occurring approximately once in 100,000 births. The presentation of this anomaly for the first time in adult life is indeed uncommon and less than 20 cases have been reported in English literature.
The primitive pulmonary system develops as an outpouching of the ventral wall of the oesophagus throughout its length. Separation of the respiratory and upper gastrointestinal tracts takes place by a septum formed from two lateral grooves. The septum becomes complete caudally first and then extends upwards into the cranial direction. Any developmental arrests or failures in the completion of this septum will obviously result in H-type TE fistula, which occurs high in the trachea . Since the oesophagus increases in length at a faster rate and more than the trachea, the fistula stretches in an upward curve with the tracheal opening more cephalad than the opening into the oesophagus.
Keeping in mind the embryological anatomy of the anomaly and the fact that a congenital 'H' type TE fistula is characteristically high (generally above the thoracic inlet) there has been a shift in surgical approach from the transthoracic to the cervical approach. A review of the different operative approaches for the repair of this anomaly reveals that the failure rate is greater when the transthoracic rather than the transcervical repair are used .
The diagnosis of the lesion still remains a challenge and unless there is a firm clinical suspicion it may be misdiagnosed. A triad of symptoms have been described which is evident if a careful clinical history is taken. These include a) chocking and coughing with feeds, especially liquids, b) history of recurrent pneumonitis or pulmonary infections, c) intermittent abdominal distnesion by air.
Accurate localisation of the level of the lesion is of utmost importance in selecting the surgical approach for its correction and repair. The diagnostic measures include an oesophagogram in prone position, tracheogram and endoscopy especially bronchoscopy. Various methods have been used to define the TE fistula intra-operatively. These include ureteral or Fogarty catheters and recently a flexible vascular guide wire passed through a fibreoptic bronchoscope preoperatively to ease identification of the fistula low down in the neck during surgery.
In conclusion, since the majority of congenital H-type fistulas are above the level of the third thoracic vertebra (T-3) they usually can be dissected for transection and repair through an incision confined to the neck. This approach has many advantages, the chief being minimum anaesthesia time and post-operative respiratory dysfunction, especially important in these patients with poor pulmonary functions secondary to recurrent aspiration.
[Figure - 1], [Figure - 2]