Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

type A

1st line – stabilization and gastrostomy

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ACUTE
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type A
1st line – 

stabilization and gastrostomy

Surgical repair of the esophagus of type A pure atresia at birth is often challenging. However, as there is no connection to the trachea, these patients are less susceptible to acute respiratory distress.

Patients may be managed with a nasogastric tube in the upper esophageal pouch providing continuous suction. Within the first few days of life, a gastrostomy tube is placed to allow for enteral feeds and the stomach contrast study to identify the length of the lower pouch.

If the length between the proximal and distal esophageal segments is 4 vertebral bodies or longer, then the patient is allowed to grow prior to attempting a repair.[7]​ Growth is monitored by using contrast radiographs, also known as a “gap study”. Typically, a probe is inserted through the gastrostomy site into the distal pouch and the nasogastric tube is advanced to the end of the proximal pouch to define the gap. When the gap between the proximal and distal segments of the esophagus is less than 2 vertebral bodies, primary esophageal repair can be performed at around 2 to 3 months of age. Originally, repair was performed via a right thoracotomy, although this procedure is now often performed thoracoscopically.

2nd line – esophageal replacement

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ACUTE
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type A
2nd line – 

esophageal replacement

In cases of failed esophageal repair or an extremely long gap, an esophageal replacement surgery is planned.[30][31] Also applies to patients who are unsuitable for surgical repair.

type B and D

1st line – suction catheter and surgical correction

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ACUTE
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type B and D
1st line – 

suction catheter and surgical correction

These patients cannot eat and are at risk for repeated aspiration of oral secretions because of the proximal fistula. Initial management is by a suction catheter in the upper esophageal pouch which should limit secretions entering the trachea. Surgery should take place in the first 24 to 48 hours of life to ligate and divide the fistula(s) and to establish esophageal continuity.

type C

1st line – stabilization and surgical correction

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ACUTE
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type C
1st line – 

stabilization and surgical correction

A nasogastric tube is placed to decompress the upper blind pouch. Positive mechanical ventilation should be avoided if possible.[17]​ The infant should be placed in a slight reverse Trendelenburg position to help prevent gastric reflux through the fistula and into the lung. Surgery should be scheduled as soon as possible.

Surgical correction is aimed at dividing the tracheoesophageal fistula to prevent lung aspiration. Anastomosis of the 2 esophageal ends is completed to establish continuity of the esophagus. Esophageal atresia/tracheoesophageal fistula (EA/TEF) repair was previously performed via thoracotomy but is now often performed using a thoracoscopic approach. Meta-analysis indicates that outcomes for either method are similar, and although the thoracoscopic procedure is associated with a longer operative time and contraindications such as severe hemodynamic instability, it may also reduce the first oral feeding time and length of hospital stay.[17]​[25]​​[26]​ The operation should be performed within the first 24-48 hours of life to decrease risk of aspiration and abdominal distension. A 2021 consortia demonstrated that the elimination of transanastomotic tubes was associated with decreased stricture rates without delaying time to initiation of per ora feeds.[27] In unstable premature infants, staged approaches such as ligation of fistula alone, or temporary occlusion of the gastroesophageal junction may be indicated. Some advocate for performing a posterior tracheopexy on all infants at the time of TEF repair whereas others perform it more selectively.[28]​ Posterior tracheopexy and rotational esophagoplasty are good techniques to use when planning surgery for a recurrent TEF.[29]

type E

1st line – nothing by mouth and surgical division of fistula

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ACUTE
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type E
1st line – 

nothing by mouth and surgical division of fistula

These patients often present later in life with evidence of choking, gagging, or recurrent aspiration.

Once diagnosed the patient should be kept nothing by mouth until the fistula is divided surgically.

Surgery is with a right neck incision at the level of the thoracic inlet. The fistula can also be reached thoracoscopically through the right chest.[32]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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