• Tracheoesophageal fistula (TEF) is typically recognized in newborns during their first feeding. Incidence is 1 in 1500–3000 live births with a slight male predominance. • Prenatally diagnosed with polyhydramnios or small stomach may be a clue to consider the presence of TEF. • In the fourth week of gestation, the esophagus and trachea separate to their respective structures from the esophagotracheal diverticulum of the foregut. TEF forms when there is incomplete division at this stage. • TEF and esophageal atresia are part of the acronym VACTERL (vertebral, anorectal, cardiovascular tracheal, esophageal, rectal, limb) describing an association of congenital anomalies. • Fistulous communication of the trachea and esophagus can result in respiratory and feeding complications. • The classic presentation of TEF is salivation, coughing, and choking with the first feeding. • Due to anatomic variants of the atretic esophagus and tracheal fistula, there are varied presentations. C is the most common, followed by A A.  Esophageal atresia without fistula (A = atresia) B.  Esophageal atresia with proximal TEF C.  Esophageal atresia with distal TEF (C = common) D.  Esophageal atresia with proximal TEF and distal TEF (D = double) E.  Tracheoesophageal fistula without esophageal atresia – Typically presents later and is associated with coughing and choking during feedings • Inability to pass an OG tube should alert clinicians to the possibility of esophageal atresia, and curling of the tube on chest XR is pathognomonic for esophageal atresia. • Additional studies with oral contrast are not recommended due to risk of aspiration. • While the esophageal atresia is problematic, the fistula connection is dangerous due to the following: A.  Acute gastric distention secondary to air entering the distal fistula connecting to the digestive system. With large gastric distension, the patient can develop rapidly progressive respiratory distress which requires emergency bedside decompression. B.  Refluxed gastric contents can also traverse the distal esophagus into the trachea resulting in aspiration. • Echocardiography and renal ultrasound are routinely performed due to possible VACTERL associations. • Differential: choanal atresia, cleft palate, esophageal ring, tracheal compression, and neurologic disorder.

Tracheoesophageal Fistula

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发表日期：

2025年

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Adam Goldsmith,
Erika B. Lindholm

摘要

• Tracheoesophageal fistula (TEF) is typically recognized in newborns during their first feeding. Incidence is 1 in 1500–3000 live births with a slight male predominance. • Prenatally diagnosed with polyhydramnios or small stomach may be a clue to consider the presence of TEF. • In the fourth week of gestation, the esophagus and trachea separate to their respective structures from the esophagotracheal diverticulum of the foregut. TEF forms when there is incomplete division at this stage. • TEF and esophageal atresia are part of the acronym VACTERL (vertebral, anorectal, cardiovascular tracheal, esophageal, rectal, limb) describing an association of congenital anomalies. • Fistulous communication of the trachea and esophagus can result in respiratory and feeding complications. • The classic presentation of TEF is salivation, coughing, and choking with the first feeding. • Due to anatomic variants of the atretic esophagus and tracheal fistula, there are varied presentations. C is the most common, followed by A A. Esophageal atresia without fistula (A = atresia) B. Esophageal atresia with proximal TEF C. Esophageal atresia with distal TEF (C = common) D. Esophageal atresia with proximal TEF and distal TEF (D = double) E. Tracheoesophageal fistula without esophageal atresia – Typically presents later and is associated with coughing and choking during feedings • Inability to pass an OG tube should alert clinicians to the possibility of esophageal atresia, and curling of the tube on chest XR is pathognomonic for esophageal atresia. • Additional studies with oral contrast are not recommended due to risk of aspiration. • While the esophageal atresia is problematic, the fistula connection is dangerous due to the following: A. Acute gastric distention secondary to air entering the distal fistula connecting to the digestive system. With large gastric distension, the patient can develop rapidly progressive respiratory distress which requires emergency bedside decompression. B. Refluxed gastric contents can also traverse the distal esophagus into the trachea resulting in aspiration. • Echocardiography and renal ultrasound are routinely performed due to possible VACTERL associations. • Differential: choanal atresia, cleft palate, esophageal ring, tracheal compression, and neurologic disorder.