Thoracoscopic repair of congenital tracheo-oesophageal fistula manifesting in an adult.

Congenital trcheo-oesophageal fistula (TOF) without oesophageal atresia is usually diagnosed and managed in the neonatal period. Its presentation in adulthood is a rarity. Traditional treatment of a TOF in adults involves its repair via a thoracotomy. We report the case of a 23-year-old man diagnosed with an H-type TOF during workup undertaken for his symptoms of gastro-oesophageal reflux. This fistula located at the level of third thoracic vertebra was repaired successfully using a thoracoscopic approach.

INTRODUCTION

Oesophageal atresia (OA) is a congenital condition of oesophageal discontinuity that results in proximal oesophageal obstruction and a tracheo-oesophageal fistula (TOF) is an abnormal communication between the oesophagus and the trachea. OA and TOF can occur alone or in combination. Congenital TOF without OA (H-type TOF) account for <4% of congenital oesophageal anomalies.[1] Late presentation of this type of TOF in adulthood is extremely rare. When detected in adults, the TOF is generally treated by open surgery. The case presented here is distinctive because of (a) the rarity of an H-type TOF in adults and (b) the utilization of a thoracoscopic approach to repair it.

CASE REPORT

A 23-year-old man was investigated for complaints of persistent night-time regurgitation for 8 years without any dysphagia. An upper gastrointestinal (UGI) endoscopy revealed a fistulous opening in the oesophagus 20 cm from the incisors [Figure 1a]. The scope passed easily through the opening into the trachea [Figure 1b]. A contrast study demonstrated the TOF and the contrast entering the right bronchial tree. A computed tomography (CT) scan with reconstruction confirmed an H-type TOF in the mid-oesophagus at T3 level [Figure 1c] and ruled out any extrinsic mediastinal pathology. A virtual bronchoscopy [Figure 1d] showed the fistula to be 3 cm above the carina. Subsequent bronchoscopy ruled out any tracheal mucosal abnormality. Oesophageal manometry demonstrated a lower oesophageal sphincter pressure to be 4.9 cm of H2 O and ineffective peristalsis in 30% of the wet swallows. The patient was counselled regarding a thoracoscopic repair of the TOF in the first instance followed by reassessment of his reflux symptoms after surgery.

Figure 1

(a) Endoscopic view of the fistulous opening in mid-oesophagus. (b) View of the carina obtained upon passing the endoscope through the fistulous tract. (c) Computed tomography scan demonstrates a tracheaoesophageal fistula. (d) Virtual bronchoscopic image demonstrating the tracheal opening (arrow)

The surgery was performed under double-lumen endotracheal anaesthesia with the patient placed in the left lateral decubitus position. A 10 mm port was established in the 6th intercostal space in the midclavicular line, and two 5 mm ports were placed on either side in the 5th and 7th intercostal space. The parietal pleura overlying the oesophagus above the level of the azygous vein was incised widely, and a pleural flap was harvested for later use. The trachea was identified, and the fistulous tract extending from the oesophagus was dissected. Vascular slings were passed to encircle the broad fistulous tract and the oesophagus distal to it [Figure 2a]. The 10 mm port was exchanged for a 12 mm Endopath Xcel port (Ethicon Endosurgery, Mumbai, India). The nasogastric tube was withdrawn, an endoscopic stapler with a blue cartridge (3.5 mm staple depth) was locked on to the fistula, and the sling isolating the fistula was removed. The stapler was fired dividing the fistulous tract [Figure 2b]. The oesophageal staple line was oversewn with a 3-0 polyglycolic acid (Vicryl, Johnson and Johnson, Mumbai, India) suture. The pleural flap was interposed between the two staple lines and sutured to the trachea in a similar manner. A 32F intercostal drain was placed through the 12 mm port site, and the incisions were closed. The operative time was 136 min.

Figure 2

(a) Sling around the fistula (F) between the oesophagus (O) and trachea (T). The second sling is around the distal oesophagus. (b) Arrow points to the tracheal end of the staple-divided fistula

Post-operatively the patient was prescribed parenteral analgesics for the first 48 h, and he was kept nil by mouth for 4 days. An esophagogram obtained on the 5th day showed smooth passage of contrast into the stomach without any leakage. The patient was allowed diet and the intercostal drain was removed. He was discharged on post-operative day 6. At follow-up a week later he was eating normally and reported significant improvement in the regurgitation. He remains well and completely asymptomatic 6 months later. It is proposed to assess his reflux symptoms a year after surgery to determine whether he would merit long-term proton-pump inhibitor therapy or anti-reflux surgery.

DISCUSSION

Benign, acquired TOFs are rare in adults and may result as a complication of inflammatory disorders, foreign body ingestion, trauma or prolonged tracheal intubation.[23] Congenital H-type TOFs presenting in adulthood are even rarer and only around 16 cases have been documented in the surgical literature.[4] The minimal symptoms in many patients may be explained on the basis of the oblique passage (an ‘N’ rather than ‘H’ configuration) of the fistulous tract leading to a valve-like action that results in the occlusion of its lumen during the passage of the bolus of food or peristaltic wave.[5] Presence of a membrane covering the opening or contraction of the of the muscle wall of the fistulous tract are considered to be the other likely explanations for the delay in onset of symptoms until adulthood.[6]

A high index of suspicion is needed to diagnose a TOF in adults, in whom the respiratory symptoms are far commoner than the gastrointestinal symptoms. The commonest complaint is cough, while eating or drinking (Ohno's sign) followed by recurrent bouts of respiratory tract infection. A lung abscess or areas of bronchiectasis may be rarely observed. Development of TOF in utero disrupts the normal development of the oesophageal myenteric plexuses resulting in dysmotility.[4] This may lead to gastroesophageal reflux (as seen in our patient) and esophagitis. A chest X-ray may show a dilated oesophagus and bilateral lung opacification. The fistulous opening is often apparent during a UGI endoscopy, but may be missed if covered with a membrane or located on the anterior wall of the upper third of the oesophagus. Similarly, the lower positioning of the oesophageal opening in relation to the one in the trachea may preclude outlining of the tract on a contrast swallow. Unless the fistula is wide (as was the case in our patient) coronal sections of the CT scan may fail to identify it. Sagittal views along with a three-dimensional reconstruction are more likely to demonstrate the fistula. Bronchoscopy is useful for identifying the fistulas not seen on UGI endoscopy and ruling out intrinsic trachea-bronchial pathology.

As in neonates, surgery is the only definitive treatment for TOF presenting in adults. Traditionally, either a cervical route (for a fistula above the T2 level) or thoracotomy (for fistulas at or below T3 level) is employed for the repair. The repair involves ligation of the fistula or its division, followed by repair of the trachea and oesophagus. A muscle or pleural flap is usually interposed between the divided ends. Over the past decade, the feasibility and safety of thoracoscopic repair of the congenital TOFs in neonates has been well documented. A recent meta-analysis comparing open and thoracoscopic surgery in the treatment of OA and TOF in neonates indicated that the outcomes of the two approaches were comparable.[7] In the only previously reported case of thoracoscopic repair of TOF in an adult, Garand et al. staple-divided a mid-thoracic level TOF in a 79-year-old female.[8] A bovine pericardial graft was interposed at the site of the transacted fistula. Thoracoscopic approach affords excellent magnification for safe visualization, dissection and division of the TOF, even when fistula is a narrow one. This complex surgery is best-undertaken either by a thoracic surgeon well versed with thoracoscopy or (as was the case here) by an experienced minimal access surgeon teamed up with a paediatric surgeon. Thoracoscopic repair of a TOF may not necessarily shorten the hospital stay, as it is customary to commence oral intake around the 5th day after confirming integrity of the staple/suture line with a contrast study. However, these patients do enjoy the other well-proven benefits of thoracoscopic surgery such as reduced pain, speedy recovery and reduction in wound-related and pulmonary morbidity.

CONCLUSION

Thoracoscopic repair appears to be a feasible and safe option for the treatment of TOF presenting in adulthood, which is an extremely rare clinical entity.