85 TRACHEOESOPHAGEAL MALFORMATIONS
Denis D. Bensard M.D., David A. Partrick M.D.

1. What are tracheoesophageal fistula (TEF) and esophageal atresia (EA)?

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The trachea and esophagus appear as a ventral diverticulum arising from the primitive foregut during the third week of gestation. The trachea and esophagus undergo separation by the ingrowth of ectodermal ridges during the fourth week of gestation. Failure of separation results in anomalous connection of the trachea to the esophagus (i.e., TEF) with or without incomplete formation of the esophagus (i.e., EA).

2. Describe the three most common variants and the relative incidence of each type

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* Proximal EA with distal TEF (”proximal pouch with distal fistula”): 85%
* Isolated EA: 10%
* TEF without EA (”H fistula”): 5%

3. What other anomalies occur with tracheoesophageal malformations?

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TEF and EA result from an insult during the critical phase of embryogenesis (3-8 weeks’ gestation). Up to 70% of infants with tracheoesophageal malformations suffer one or more concomitant anomalies. Cardiovascular anomalies are the most prevalent (35%), followed by anomalies of the gastrointestinal (24%), genitourinary (20%), skeletal (13%), and central nervous (10%) systems. Twenty-five percent of infants born with tracheoesophageal malformation have one or more components of the VACTERL association (see question 2 in chapter 84).

4. Does the presence of other anomalies alter management and outcome?

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Healthy infants without concomitant anomalies generally undergo early repair with a nearly 100% survival rate, whereas infants who are severely premature or have life-threatening anomalies typically undergo delayed repair. Infants with lethal anomalies, such as trisomy 18, receive palliative care only.

5. Describe the clinical presentation, diagnosis, and preoperative management of patients with EA with distal TEF.

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Early in the newborn period, affected infants demonstrate excessive salivation (i.e., inability to swallow secretions), choking, or regurgitation with feeding (i.e., inability to swallow feeds). Respiratory distress quickly ensues because of aspiration of secretions or feeds from the esophageal pouch and reflux of gastric acid into the airways and lungs via the distal TEF. A nasogastric tube cannot be advanced into the stomach. The radiograph demonstrates a blind-ending proximal esophageal pouch and an air-filled stomach caused by the anomalous connection of the distal esophagus to the airway. The infant is maintained in a semi-upright position with sump catheter drainage of the proximal esophageal pouch to minimize contamination of the lungs either because of aspiration or reflux.

6. Describe the clinical presentation, diagnosis, and preoperative management of isolated EA.

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Isolated EA is associated with excessive salivation, choking, and regurgitation of feeds. The inability to pass a nasogastric tube into the stomach and a gasless abdomen apparent on radiograph suggests the diagnosis. Preoperative management is directed to the identification of associated anomalies and determination of gap length. Sump catheter drainage of the proximal esophageal pouch is maintained to minimize aspiration. Gastrostomy is generally performed within the first 24 hours of life to permit feeding and assessment of the distal esophageal length. Typically, infants with EA undergo delayed repair to permit growth of the distal esophagus and reduction of gap distance.

7. Describe the clinical presentation, diagnosis, and preoperative management of TEF without EA.

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These infants demonstrate repeated choking or cyanotic spells with feeding caused by the reflux of feeds from the esophagus to the lungs via the anomalous tracheoesophageal connection. Older infants and children may present with recurrent bouts of pneumonia or unexplained reactive airway disease resulting from the intermittent contamination of the lungs via the fistula. Video esophagography and bronchoscopy are used to demonstrate the fistula.

8. How are tracheoesophageal malformations corrected surgically?

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Surgical treatment entails restoration of esophageal continuity and elimination of the pathologic connection of the esophagus to the airway. Correction of EA with or without TEF requires thoracotomy, with or without ligation of TEF, and end-to-end esophageal anastomosis. The first successful procedure was performed by Cameron Haight in 1941. At 5-7 days after surgery, an esophagogram is performed; if no leak is visualized, oral feedings are started and the pleural drain is removed.
TEF without EA is approached via a cervical incision, avoiding thoracotomy. The fistulous tract is divided and healthy tissue is interposed to prevent recurrence.

9. What are the early and late complications of surgical repair?

Early complications

Early complications are related to the basic surgical principles of wound healing. Anastomotic disruption generally results from poor blood supply and tension.
Late complications

Most strictures (50%) respond to one to three dilatations performed in the first 6 months of life. Refractory strictures require identification of associated gastroesophageal reflux (GER), which may worsen stricture formation. The frequency of GER appears related to gap length (i.e., the greater the gap distance, the greater the risk of significant GER).
KEY POINTS: TRACHEOESOPHAGEAL MALFORMATIONS

1. The three most common variants are proximal esophageal atresia (EA) with distal tracheoesophageal fistula (TEE), isolated EA, and TEF without EA.
2. Early in the newborn period, affected infants demonstrate excessive salivation, choking, or regurgitation with feeding.
3. Surgical treatment entails restoration of esophageal continuity and elimination of the pathologic connection of the esophagus to the airway.

References
BIBLIOGRAPHY
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