Congenital tracheo-esophageal fistula

Changed by Tony Chen, 8 Oct 2015
Back to revision history

Updates to Article Attributes

Body was changed:

Congenital tracheo-oesophageal fistula is a congenital pathological communication between the trachea and oesophagus.  

Demographics and clinical presentation

Tracheo-oesophageal fistula and oesophageal atresia have a combined incidence of approximately 1 in 3500 liver birthslive births 1-3,5. There is only a minimal hereditary/genetic component with incidence in twins and those with family history being only approximately 1% 5. There is no convincing gender or racial predilection 5.

Clinical presentation is similar for all types except for type E (H-type) which is the odd-man out.

Antenatally polyhydramnios may be present due to inability to adequately swallow fluid, and is found in up to 90% of cases 1, but is unfortunately a non-specific sign.

The diagnosis is usually made in the neonate, as they experience feeding difficulties and respiratory compromise due to repeated aspiration. In cases where oesophageal atresia is present (i.e again, all but H-type), then attempted passage of a nasogastric tube will not be successful. Only with H-type fistulae, which can be very small, and typically slant down from the trachea toward the oesophagus, may presentation be delayed, sometimes by a number of years 2.

Associations

In just over half of cases, there are other associated congenital abnormalities, including1-2,5:

Pathology

The trachea is an out-budding from the ventral foregut, and tracheo-oesophageal fistulae represent incomplete/abnormal division. They are very closely related to oesophageal atresia, and represent a spectrum of disease. As such, the types of oesophageal atresia/tracheo-oesophageal fistula can be divided into 1-2:

  • proximal atresia with distal fistula: 85%
  • isolated oesophageal atresia: 8%
  • isolated fistula (H-type): 4%
  • double fistula with intervening atresia: 1%
  • proximal fistula with distal atresia: 1%

See oesophageal atresia classification.

Radiographic features

Ultrasound

Antenatal ultrasound may demonstrate polyhydramnios or even in some cases a distended proximal blind ending oesophagus 1.

Plain film 

Demonstration of the nasogastric tube curled in the proximal oesophagus in a child where passage of the tube has been unsuccessful is usually sufficient for diagnosis. The proximal oesophageal stump may be distended with air (types A and C).

The presence of air in the stomach and bowel in the setting of oesophageal atresia implies that there is a distal fistula.

Often the lungs demonstrate areas of consolidation/atelectasis due to recurrent aspiration. 

Fluoroscopy (Barium studies)

H-type fistulas can be difficult to diagnose and may require contrast studies, looking for contrast passing into the tracheo-bronchial tree.

CT

In some instances CT and virtual bronchoscopy may be of benefit in preoperative planning 4. This is especially the case in patients with long segment atresia who may require a staged operation.

Treatment and prognosis

Prior to surgical correction, bronchoscopy is frequently performed. In infants who are deemed unable to tolerate emergency repair, then a gastrostomy and sump drainage catheter may be performed to allow feeding and weight gain. Another reason for delay is in patients who have a 'long-gap' atresia precluding primary anastomosis repair. In most cases however the defect is repaired immediately. 

Prognosis is often most affected by the presence of associated congenital anomalies. For example in one study (albeit from 1979) the mortality for infants with just oesophageal atresia/tracheo-oesophageal fistula was 23% versus 79% in those with associated cardiac anomalies 3.

See also

  • -<p><strong>Congenital tracheo-oesophageal fistula</strong> is a congenital pathological communication between the trachea and oesophagus.  </p><h4>Demographics and clinical presentation</h4><p>Tracheo-oesophageal fistula and <a href="/articles/oesophageal-atresia">oesophageal atresia</a> have a combined incidence of approximately 1 in 3500 liver births <sup>1-3,5</sup>. There is only a minimal hereditary/genetic component with incidence in twins and those with family history being only approximately 1% <sup>5</sup>. There is no convincing gender or racial predilection <sup>5</sup>.</p><p>Clinical presentation is similar for all types except for <a href="/articles/eesophageal-atresia-classification">type E (H-type)</a> which is the odd-man out.</p><p>Antenatally <a href="/articles/polyhydramnios">polyhydramnios</a> may be present due to inability to adequately swallow fluid, and is found in up to 90% of cases <sup>1</sup>, but is unfortunately a non-specific sign.</p><p>The diagnosis is usually made in the neonate, as they experience feeding difficulties and respiratory compromise due to repeated aspiration. In cases where oesophageal atresia is present (i.e again, all but H-type), then attempted passage of a nasogastric tube will not be successful. Only with H-type fistulae, which can be very small, and typically slant down from the trachea toward the oesophagus, may presentation be delayed, sometimes by a number of years <sup>2</sup>.</p><h5>Associations</h5><p>In just over half of cases, there are other associated congenital abnormalities, including<sup>1-2,5</sup>:</p><ul>
  • +<p><strong>Congenital tracheo-oesophageal fistula</strong> is a congenital pathological communication between the trachea and oesophagus.  </p><h4>Demographics and clinical presentation</h4><p>Tracheo-oesophageal fistula and <a href="/articles/oesophageal-atresia">oesophageal atresia</a> have a combined incidence of approximately 1 in 3500 live births <sup>1-3,5</sup>. There is only a minimal hereditary/genetic component with incidence in twins and those with family history being only approximately 1% <sup>5</sup>. There is no convincing gender or racial predilection <sup>5</sup>.</p><p>Clinical presentation is similar for all types except for <a href="/articles/eesophageal-atresia-classification">type E (H-type)</a> which is the odd-man out.</p><p>Antenatally <a href="/articles/polyhydramnios">polyhydramnios</a> may be present due to inability to adequately swallow fluid, and is found in up to 90% of cases <sup>1</sup>, but is unfortunately a non-specific sign.</p><p>The diagnosis is usually made in the neonate, as they experience feeding difficulties and respiratory compromise due to repeated aspiration. In cases where oesophageal atresia is present (i.e again, all but H-type), then attempted passage of a nasogastric tube will not be successful. Only with H-type fistulae, which can be very small, and typically slant down from the trachea toward the oesophagus, may presentation be delayed, sometimes by a number of years <sup>2</sup>.</p><h5>Associations</h5><p>In just over half of cases, there are other associated congenital abnormalities, including<sup>1-2,5</sup>:</p><ul>
  • -<li><a href="/articles/edward-syndrome">trisomy 18</a></li>
  • +<li><a href="/articles/edwards-syndrome-1">trisomy 18</a></li>

ADVERTISEMENT: Supporters see fewer/no ads