A tale of two tubes: Type E tracheoesophageal fistula

A one-year-old girl, born of a non-consanguineous marriage, presented with multiple episodes of cough, fever and regurgitation of feeds with resultant recurrent aspiration pneumonitis since one month of age. Examination findings showed grunting, stridor and coarse crepitations in the left lower zone. She underwent an oesophagogram for the same indications.

Pre-operative barium (95% w/v) oesophagogram

On the initial posteroanterior (PA) chest view, there was an area of increased opacity consistent with consolidation, located at the left perihilar and left retrocardiac region. The tip of the nasogastric tube was at the pylorus (Figure 1a). The oesophagogram showed a fistulous communication measuring 2.8 x 1.4 mm between the trachea and the oesophagus at the level of the T1–T2 vertebral disc with aspiration of contrast into the tracheobronchial tree bilaterally (Figure 1b). Features were suggestive of type E, “H” shaped, tracheoesophageal fistula with left lower lobe consolidation secondary to aspiration.

Post-operative barium (95% w/v) oesophagogram

On the PA chest view, there was a linear opacity at the left lower lung field, consistent with subsegmental residual atelectasis (Figure 2a). The oesophagogram showed unobstructed passage of the barium into the stomach. There was no aspiration of barium into the tracheobronchial tree or mediastinal leak (Figure 2b). There was a 2 x 2 mm outpouching from the right anterolateral wall of the oesophagus at the C7 level (Figure 2c). Features were suggestive of an oesophageal diverticulum at the position of the ligated fistula.

Tracheoesophageal fistula (TEF) is an abnormal communication between the trachea and the upper oesophagus. When acquired, the most common causes are prolonged intubation, oesophageal malignancy, iatrogenic or accidental trauma and granulomatous mediastinal lymphadenopathy (tuberculosis) [1]. When congenital, it occurs due to failure of separation of the ventrally developing respiratory bud from the dorsal foregut [2]. TEF can be part of single gene disorders like CHARGE syndrome and Pallister–Hall syndrome, trisomies 13, 18 and 21, and seen with VACTERL association [3].

There are five types of TEF with or without concomitant oesophageal atresia. The most common is type C [4], as illustrated in Figure 3. In types A, B, C and D, oesophageal atresia presents in the neonatal period with excessive drooling of saliva and regurgitation of feeds, and a history of polyhydramnios in the antenatal period. In types B and D, there may be respiratory distress due to aspiration pneumonitis. In our case, the infant presented late due to the absence of oesophageal atresia. She had a history of recurrent bouts of aspiration pneumonitis, as commonly seen in children with TEF type E [5].

In types A, B, C and D, the diagnosis can be suspected by the inability to pass an infant feeding tube/nasogastric tube till the stomach, with resultant coiling in the proximal atretic oesophagus and an absent fundic bubble on a chest radiograph in types A and B [6]. The most useful investigation is the oesophagogram with water-soluble contrast. In our case, due to the large diameter of the fistula, it was easy to identify a contrast leak into the trachea in the supine position. However, if the fistula diameter is smaller, it can be inconsistently patent and may result in a false negative oesophagogram. In such cases, operators should scan the infant in the prone position with a horizontal x-ray beam and inject contrast with very careful volume control through a nasogastric tube, which should end at the mid-oesophagus until the upper oesophagus is distended with contrast. Recording the fluoroscopy video for the surgeon is recommended. Radiologists should pay attention not to misinterpret oesophageal overflow resulting in laryngeal aspiration for a fistula. If unsuccessful,  other procedures like bronchoscopy with methylene blue injection or CT scan with virtual bronchoscopy can be employed. TEF type E can be depicted with a single contrast oesophagogram in the right lateral to right anterior oblique position with full distension of the oesophagus [7].

TEFs type E are managed by Vicryl/PDS ligation of the fistula and non-tension closure of both ends by a right cervical approach, as in our case. If the fistula occurs caudal to the D2 level, a thoracotomy is performed. The most common long-term problems include vocal cord injury and gastroesophageal reflux [8]. Post-operative complications include anastomotic site stricture, anastomotic site leak and recurrence of fistula in decreasing order of frequencies [9]. All of these complications can be diagnosed on oesophagograms with barium as a contrast medium. In our case, the post-operative oesophagogram showed no leak or stricture at the fistula site. However, an anterior diverticulum was demonstrated, representing a remnant of the oesophageal bud component of the ligated fistula.