Clinical Syndromic Groups in Patients with a Congenital Tracheoesophageal Defect

A patient with a trachesophageal defect may present with signs and symptoms that correlate with the type of defect present.

Signs and Symptoms	Clinical Group	Probable Anatomic Correlate
excessive salivation	I	esophageal atresia (without tracheal fistula)
excessive salivation coughing and choking cyanosis	II	esophageal atresia with tracheal fistula to distal esophagus
coughing and choking at feeding cyanosis at feeding	III	esophageal atresia with tracheal fistula to proximal esophagus
episodic coughing and choking episodic cyanosis	IV	tracheal fistula to esophagus without atresia
partial regurgitation and dysphagia	V	esophageal stenosis (without tracheal fistula)

Presentations:

(1) The first 3 groups usually present in infancy and would be associated with poor weight gain and other symptoms.

(2) Patients in Group IV tend to have problems as infants, but problems can persist into adulthood.

(3) Patients in Group V may go undetected if stenosis is mild.

Patterns:

(1) excessive salivation: atresia of esophagus with upper segment ending blindly

(2) coughing and choking: tracheal fistula

(3) cyanosis:tracheal fistula

(4) symptoms associated with feeding: esophageal atresia with tracheal fistula arising off upper segment

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